PEDIATRICS

EXAMINATION REVIEW

Questions , Answers And Teaching nots for each question
Dr. kamel hassan
C.E.S DE PEDIATRIE, C.P.B. OF PEDIATRICS Medical Services Directorate

PEDIATRICS EXAMINATION REVIEW

This book acknowledges those who anti cipate the need to use this book and those who made it possible. It is dedicated to the Palestine medical council and all my colleagues.

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PEDIATRICS EXAMINATION REVIEW

INTRODUCTION
This is a test book, not a text book. This test book will be a learning experience because the questions and answers are structured to give you experience in preparation for any examination. In addition ,the questions and answers are structured to teach a great deal about problems, disease, differenti al diagnosis, process, and therapy. It will test your medical skills and approach to diagnosis, laboratory confirmation, therapeutic planning, and anticipated outcome. Pediatrics has been substantially revised and updated to keep in step with current trends in medical education and the continuing expansion of scientific knowledge. The questions themselves are organized in broad categories to give you a representative sampling of the material covered in course work and are modeled as closely as possible after those used by the board. The answers and teaching notes follow each questions. Using this test book, you may identify the areas of strength and weakness in your command of the subject.

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PEDIATRICS EXAMINATION REVIEW

Questions
(PART 1)

TRUE OR FALSE

4

Is maintained using a depolarising agent such as suxamethonium. 4. 6. 2.Muscle relaxation during surgery: 1. Shows a linear increase with PaO2. 5. The presence of HbsAg indicates acute infection only 5. Lead to excess stimulation of T cell proliferation and interleukin-2 production 4. Nitrofurantoin 4. 5. Block acetyl-choline receptors on muscle end plates. 3. 4. 3.Causes of pulmonary fibrosis include: 1. HBsAg is the first serological marker to appear 4. Is unaffected by temperature. Methysergide 5.The following statements are true regarding superantigens: 1. HBV only replicates in the liver 3. Right-sided aortic arch. 2. 5 .Regarding hepatitis B virus (HBV): 1. The persistence of HbeAg indicates infectivity 2. Lead to stimulation of production of TNFa and other cytokines 3. HBV is an RNA virus 2.PEDIATRICS EXAMINATION REVIEW 1. They are very strongly antigenic bacterial polysaccharides 2. They bind to B cell receptors 3.The following are clinical associations of Fallot’s tetralogy: 1. 4. Digital clubbing. Penicillamine 4. Cerebral abscess. Chlorpromazine 3. They are responsible for gram-negative toxic shock 5. Single second heart sound. Sulfasalazine 2. 5. 5. 2. Shows a right shift in the autoregulation curve in patients with chronic hypertension. Allows improved intra-abdominal access. Side effects include histamine release. Is decreased at 72 hours in a normal person who is made to hyperventilate for 72 hours. 3. Is increased in an acutely compensated metabolic acidosis.Cerebral blood flow: 1. Is not necessary in ventilated patients. Aortic ejection click.

12-The following can cause card iomyopathy in HIV disease: 1. there is near -complete absorption of glucose and amino acids 5. Kaposi Sarcoma 4. Is associated with high intelligence 3. 5. whi ch of the followings are true? 1. In patients over 40 years of age. Can be inherited 5. Is defined as Head circumference > 1 SD above mean 2. In nephrotic syndrome. The GFR of a child reaches adult values (corrected for weight) by the age of one year 2. 3. In the normal kidney. Reidel's thyroiditis. The fluid entering the DCT is iso-osmolar 3. They are highly protein bound drugs 5. 2. Motor neurone disease. There are approximately one million nephrons in each kidney 11-The following conditions occur due to the presence of circulating antibodies: 1. due to an increase in bradykinin levels 3. Is associated with neurofibromatosis 10-Regarding normal renal physiology: 1. the loss of immunoglobulin in urine is the only cause of predisposition to infection 2. Hashimoto thyroiditis. Is associated with Cornelia de Lange syndrome 4. will cause an increase in renal blood flow 4. 4. Under the influence of ADH. Selective IgA deficiency is the commonest inherited form 4. Graves disease. They cause hypotension via a drop in systemic vascular resistance 2. a chest radiograph is mandatory to exclude thymoma 5. there is an increased incidence of lymphoma 3. In the proximal tubules. May lead to a side effect of dry cough.ACE inhibitors: 1. CMV infection 5. Lymphoma 3. Mycobacterium tuberculosis 2. The genetic defect in X-linked agammaglobulinaemia (XLA) is in Bruton’s tyrosine kinase (Btk) 8. In common variable immunodeficiency.PEDIATRICS EXAMINATION REVIEW 7-In hypogammaglobulinaemia. HIV of the heart 6 . Myasthenia gravis. Indications include congestive cardiac failure in a patient with renal artery stenosis 9-Macrocephaly :1. there is increased permeability to water in the DCT 4.

Baroreceptors are the principal sensors for controlling sodium secretion and reabsorption. In reduced sodium states. Hypoxia-induced vasoconstriction is the mechanism of closure of the ductus arteriosus 3. Lungs secrete angiotensinogen. Is due to a failure of Ig isotype switching 16. Production begins at 30 weeks 2. Is associated with normal levels of IgG 3.The following is true regarding changes in the fetal cardiovascular system after birth: 1. 17. Inferior vena caval pressure falls after birth 15.X-linked hyper IgM syndrome :1. Prolonged general anaesthesia 3. Production is increased during a stressful event like hypothermia 5.Regarding surfactant:1. both increased activity of renal sympathetic nerves (leading to constricting afferent arterioles) and reduced glomerular capillary pressure.Gastric emptying is slowed by: 1. 3. Supine posture 2. Multiple MRI scans 18. The ligamentum teres is the remnant of the umbilical vein 4. Domperidone 4. Is due to a defective CD40 ligand 5. Gastric distention 3. kidneys secrete renin. 4. 2. liver cells contain angiotensin -converting enzyme. Regression of right ventricular hypertrophy occurs postnatally 5. Massive blood transfusion 4. There is normally immediate closure of the ductus arteriosus 2. Ranitidine 5. Testosterone stimulates surfactant production 4.Control of absorption: Which of the following statements are true? 1. Causes defective B cell development 2. It is produced by Type II pneumocytes in the walls of the bronchi 3. Systemic steroids 2.PEDIATRICS EXAMINATION REVIEW 13. decrease Na+ and water excretion. Cisapride 14. 5. Can present with pneumocystis carinii infection 4. Total body irradiation 5. Changes in aldosterone levels allow control of the re -uptake of 50% of sodium originally present in the glomerular filtrate. Betamathasone given to the mother improves surfactant production in the premature baby 7 . Renin secretion is controlled by the macula densa.A cataract may be induced accidentally by the following treatments: 1.

Normal children 2. Tall P waves are seen on ECG 2. Live vaccines are contraindicted within 3 weeks of another live vaccine administration 2. Contains salts that are only water soluble 5. Drash syndrome 3. Calcium gluconate exchanges for potassium and enables its excretion 5. Severe febrile illness is a contraindication for DPT 5. Pulmonary hypertension 23. Are derived from a precursor cell in the bone marrow 2. Noonan's syndrome 5.T lymphocytes:1.The following syndromes commonly have associated renal manifestations: 1. 95% die in the thymus 3. Are prominent in type II hypersensitivity reactions 8 . Is secreted by the bile canaliculi 2.Wide splitting of second heart sound may be evident in: 1. MMR can be given a week after administration of IV immunoglobulins 3.The following are true regarding vaccine administration in children: 1. Chromosome 11p deletion 20. Is released from the gall bladder in response to the hormone gastrin 4.PEDIATRICS EXAMINATION REVIEW 19. If acidosis is corrected.Regarding hyperkalaemia : 1. Meckel Gruber syndrome 4. Live vaccines are not given to children on prolonged steroid therapy 4.Bile: 1. Volume overload due to partial anomalous venous drainage (PAPVD) 3. All have the alpha-beta heterodimeric T cell receptor 4. Severe aortic defect 4. IV calcium resonium should be commenced 4. Severe malnutrition is a contraindication for BCG 22. Continuous salbutamol nebulisers should be started immediately on diagnosis 3. Zellwegers syndrome 2. Atrial septal defect 5. potassium shifts back into the intracellular fluid 24. Salts are mostly reabsorbed in the large bowel 21. Contains NaHCO3 3. Are usually activated by intact protein antigens 5.

PEDIATRICS EXAMINATION REVIEW 25. Is associated with feelings of detachment 26. 4. 3.Disseminated intravascular coagulation: 1. 2.000. Progresses to dilated cardiomyopathy in 10% cases 5. It may be associated with aortic stenosis. It can present in the newborn. 3. Is characterised by arterial hypertension. Is usually associated with a normal thrombin time 3. 9 . May be associated with formation of microthrombi. The prognosis is related to the degree of left ventricular outflow gradient 2. The central venous pressure is raised. Should be treated with aspiration using a needle inserted via the second intercostal space. 5. The incidence is about 1:10. 5. Is inherited as an autosomal dominant disorder 2. The genetic mutation of type I has been linked to chromosome 16. Is an immediate response to a traumatic event 2. May occur in liver disease 5. May lead to avoidance 4.Post-traumatic stress disorder : 1. Is one of the causes of retinitis pigmentosa 5. Prognosis is worse in children 3. May be associated with cytomegalovirus or varicella zoster infections 4. mid-clavicular line. Is characterised by increase ECG voltage across all leads. Is usually associated with a high fibrinogen level 2. Is associated with icthyosis 3. Is caused by the same enzyme as Hurler’s disease 4. 28-The following statements are true regarding Hypertrophic obstructive cardiomyopathy:1. Strenuous activity is proscribed in all patients 29. Occurs only after penetrating injuries to the heart. Runs of Non sustained ventricular tachycardia on Holter have a high predictive value for sudden cardiac death 4. Is affected adversely by radical weight change 27. 4.Refsum’s Disease: 1. 2.The following are true of adult polycystic kidney disease: 1. so should be treated with intravenous heparin 30-Cardiac tamponade: 1. Liver cysts are seen in approximately 10%. Can sometimes be treated by play therapy 5. Often involves ‘flashbacks’ 3.

Neonatal infection is more likely if the mother has concomitant HIV infection as well as HCV 4. Infertility is a frequent complication of the orchitis associated with mumps 5. Cause peripheral dilatation 4.PEDIATRICS EXAMINATION REVIEW 31-Regarding glycogen and its metaboli sm: 1. Cause relaxation of the diaphragm 5. Hamoglobinuria is a characteristic finding 3. Thalassaemia 3. Liver transplantation is usually required due to the inadequacy of medical therapies 33-Which of the following conditions would be suitable for somatic gene therapy: 1. Adenosine deaminase deficiency 2. The diagnostic test is Ham’s test 5. Sickle cell disease 34-In paroxysmal nocturnal haemoglobinuria: 1. McArdle’s Disease results in fasting hypoglycaemia and hepatomegaly 32-Concerning Hepatitis C Virus: 1.4 -glycosidic linkages 3. Have an anti-diuretic effect 2. Transforms into acute leukaemia in approx. 1% of the world’s population is infected 2. The defect is a congenital abnormality of RBC production by the bone marrow 2. It is often asymptomatic 3. Venous thrombosis is a recognised complication 4. Breast feeding should be discouraged in the HCV +ve mother 5. The incubation period is 7-14 days 2. Branches in the glycogen molecule are produced by 1. Deficiency of muscle phosphorylase. Cause bronchodilation 3. It is caused by a DNA virus 10 . Children are not infectious after the development of parotitis 4. may result in an impaired exercise tolerance 5. The proportion developing chronic infection and hepatocellular carcinoma are similar to HBV infection 3. Haemoplilia A 5. Glycogenolysis in the liver releases glucose-6-phosphate into the blood stream 2. Diabetes mellitus 4. 5% 35-Phosphodiesterase inhibitors: 1. Decreases the pulmonary capillary wedge pressure 36-Regarding mumps infection: 1. Adrenaline is an important stimulus for glycogenolysis in the liver during exercis e 4.

Organ rejection is an early post operative complication 40-The following statements are correct regarding Horner’s syndrome: 1. 3. Production of suppressive cytokines such as IL -10. May be associated with ophthalmoplegia in the territory of the IIIrd cranial nerve. Maximal expiration 3. It may be caused by injury to the superior cervical sympathetic ganglion. rubella and polio are contraindicated in HIV +ve children 4. Pupillary dilatation occurs because of loss of innervation to the iris sphincter muscle. 41-In HIE or Job’s syndrome : 1. May be associated with loss of sweating on the ipsilateral arm. “Cold abscesses” occur 42-The following increases the pulmonary vascular resistanc e 1. Prostacyclin 11 . Ptosis occurs because of loss of innervation to the smooth muscle portion of the levator palpebrae superioris. mumps. Maximal inspiration 2. 2. FK506 has superceded tacrolimus as an immunosuppressant 4. There is a low incidence of atopy due to defective IgE function 4. Anemia 4.PEDIATRICS EXAMINATION REVIEW 37-The following are known functions of T helper cells : 1. Prolonged inconsolable crying to previous immunisation is a definite contraindication. The condition is X-linked 5. Measles. Production of perforin 4. kyphoscoliosis. 5. There is an association with coarse facies. Production of T cell growth factors such as IL-2 2. There is a markedly raised IgE level 2. Induction of the maturation of immature dendritic cells 38-Regarding immunisation in the UK: 1. 4. and eczema 3. Signs of chronic liver disease persist despite improvement in liver function 5. TGF-beta 5. 39-Regarding paediatric liver transplantation:1. The first dose of vaccines is given at a corrected age of 2 months in case of premature delivery 2. Family history of febrile convulsion following immunisation is a relative contraindication 3. osteoporosis. Promotion of immunoglobulin isotype class switching 3. Salbutamol 5. 5-yr survival rates for children after orthotopic liver transplantation are around 30% 3. The most common indication is failed Kasai procedure 2. Live vaccines should be avoided within 6 months of termination of lymphoma treatment 5.

Leukotriene inhibitors are associated with the development of Churg-Strauss . cellulose and glycogen are broken down for absorption by salivary and pancreatic amylases. About 9 litres of water enters the gut each day. Amitriptyline 4. Cefoxitin 4. The three known fat soluble vitamins A. 5. Should be treated aggressively in the early stages of the disease 4. 5. Local humidity and air movement do not significantly influence heat loss. Is asymptomatic at diagnosis in les s than 10% of patients 3. 46-In asthma:1. The skin is a good thermal insulator whose effectiveness is controlled through blood flow. Changes in muscle activity are the main source of heat production for temperature control. haemodialysis would be effective in removing drug in: 1. 47-Regarding overdose. Benzylpenicillin 2.5 litres per day and eliminate 2. Digoxin 2. Has a median survival of less than 5 years 5. 12 . 4. Metronidazole 5. carboxypeptidases and amino peptidases are responsible for the breakdown of proteins for absorption. Piperacillin / tazobactam 3. Salicylate 48-Heat inputs and outputs: Which of the following statements are true? 1. Is associated with smear or smudge cells in the peripheral blood 2. Pethidine 3. D and E are taken up by fat absorbing processes in the gut. The glucose-containing polysaccharides starch. Blocking interleukin-5 maybe therapeutic 4. chymotrypsin. Sweating can increase body evaporative water loss from 0. Is associated with splenomegaly at diagnosis in about 50% of patients 44-The following agents have good activity against Bacteroides fragilis : 1. 3. 3. The eosinophils to the lung are recruited from the bone marrow 2. 4. 2. Propranolol 5. 2. Chloramphenicol 45-Digestion and absorption: Which of the following statements are true? 1. The blood flow through skin is controlled through vasodilator sympathetic nerves whose firing rate is increased in response to heat.6 to over 4. Bronchial hyper-responsiveness (BHR) does not have to be present. Amphipathic phospholipids and bile salts convert micelles into lipid droplets ready for absorption. Trypsin. Steroids are contra-indicated if the patient is a diabetic 3.PEDIATRICS EXAMINATION REVIEW 43-Chronic lymphocytic leukaemia : 1. 5.4 Mcal heat from the body.

4. Exacerbations may be associated with lymphadenopathy 2. Which one of the following investigations is the most relevant for her future management? 1. She is the only child of her parents. Chromatin plays no role. It is treated b y routine nephrectomy 3. Serum adrenocorticotrophic hormone (ACTH)/cortisol 4. There is an increased liability to infections 3.P was concerned about this and has addressed this i ssue with her and her parents. Is associated with an increased risk of ischaemic heart disease 53-In scleroderma (systemic sclerosis): 1. Only 10% of affected pregnancies have resulting long term sequele at birth 2. pigmented rash on her neck and axilla. Fasting serum cholesterol/triglyceride 2. Transcription factors control expression by binding to specific DNA sequen ces. UnilateraI lesions have an incidence of 1:2500 -1:4000 live births 4. Diagnosis is by viral isolation from the urine 3.P with a velvety. Penicillamine may delay the development of internal organ complications 54-Regarding multicystic dysplastic kidney disease: 1. There is usually 0% differential function on DMSA/ MAG3 imaging 5. 3. Hearing loss can deveolp gradually over the first 5 years 4. DNase I hypersensitivity sites are usually indicative of regions of active chromatin. Calcinosis only occurs in CREST form of the disease 4. Females are affected three times more commonly than men 3. 2. Transcription levels determine protein levels.PEDIATRICS EXAMINATION REVIEW 49-In gene regulation: 1. Serum thyroid-stimulating hormone (TSH) 51-Congenital CMV infection: 1. Raynaud’s phenomenon is the commonest presenting symptom 5. Fasting serum insulin/glucose ratio 3. Hypertension is a common complication of MCDK that warrants nephrectomy 13 . It is not itchy and she is not disturbed by it. The affected newborn should be treated with ganciclovir 5. Her weight is 49 kg and her G. Plasma leptin 5. It is a rare anomaly that presents as an abdominal mass in the neonate 2. 5. Anti-Scl 70 antibodies occur in 50-60% of cases 5. The stability of mRNA is entirely dependent on its length. 50-Sarah is eight years old and she wa s referred by her G. Intracranial calcifications are seen in a periventricular distribution 52-In systemic lupus erythematosis (SLE) 1. Intravenous immunoglobulins are commonly used in severe disease 4. Skin tightness may improve with time 2.

Arterial CO2 rises during sleep 5. Is transported in the blood as phosphate esters 3. Does not affect plasma HCO3 concentration 2. urticaria and eosinophilia may be seen in the initial illness due to antigen -antibody complex formation 4. Schistosoma haematobium infection may lead to an obstructive uropathy 56-Regarding platelets: 1. Infection invariably occurs following oral ingestion of contaminated food products 3. Is transported in the blood in simple solution 4. Results in a non-invasive enteric infection 2. Quinine 3. Fever. Is surgically treated by excision of the pylorus muscle 60-The following are recognised causes of dr ug-induced immune thrombocytopenia:1. They have a life span of approximately 10 days 2. Platelets can be stored for a maximum of 48 hours 5. Penicillamine 4. Presents with projectile. Schistosoma haematobium infection may lead to rectal bleeding 5. Freshwater snails are the definitive host 2. Antibiotic treatment is required for Salmonellosis 5. bilious vomiting 4. Rifampicin 5. Platelets should be stored between 2 and 6 degrees C 4. Is more common in the Afro-Caribbean population 3. potassium and bicarbonate 5.PEDIATRICS EXAMINATION REVIEW 55-Regarding schistosomiasis: 1. Platelet production is controlled by specific cytokines 3. Chloramphenicol 2. A pool of platelets transfused can produce an increment of 50 000 / ml if platelet consumption is not an issue 57-Concerning Salmonella infection: 1. Results in biochemical changes including low serum chloride. Heparin 14 . Cercariae penetrate the skin and may lead to an itchy papular rash 3. With typhoid 15-20% become chronic carriers 58-Carbon dioxide: 1. Arterial CO2 rises when living at an altitude of 9000 meters 59-Infantile hypertrophic pyloric stenosis: 1. Statutory notification is required 4. Is more common in male babies than female babies 2.

Transposition of the great vessels 3. Transparent hyaline casts are nonspecific and may be present in any case of ARF unrelated to the specific aetiology. 4. 4. 3. Core temperature <35 deg. Fallot's tetralogy 5. Ciprofloxacin is used for routine contact prophylaxis 63-Features of the systemic inflammatory response syndrome (SIRS) include: 1. 62-Concerning meningococcal disease: 1. Frequently co-presents with meningitis. Is a syndrome associated with chronic inflammation of peripheral nerves.cent. Pigmented 'muddy brown' granular casts suggest acute tubular necrosis. 3.PEDIATRICS EXAMINATION REVIEW 61-The following statements are correct regarding urinary analysis in acute renal failure: 1. 64-The stiff -man syndrome :1. Respiratory rate >16/min. Is uniquely associated with GAD antibodies. 4. Are associated with cataracts 4. Develop hemiplegia on the ipsilateral side of the vascular lesion 3. Mannose binding protein deficiency is a known risk factor for meningococcaemia 5. 5. Is best treated by benzodiazepenes and baclofen. Is readily distinguished on clinical grounds from other infectious agents by the spreading purpura 2. Presents in seasonal epidemics 3. Hypercapnia with PCO2 >6 kPa. 2. 3. Occurs in 50% of children with port wine stains in the ophthalmic division of the trigeminal nerve 2. 66-Sturge-Weber syndrome :1. Typically have slit like ventricles on CT 5. 2. RBC casts suggest primarily renal arteriolar diseases such as vasculitis. There is a good correlation between functional renal impairment and the findings on urinalysis. making LP necessary at presentation 4. 5. Tachycardia >90 bpm. Presence of proteinuria argues against acute tubular necrosis and suggests a primary gomerular pathology. Is associated with fibrillation and myokimeia. Fallot's pentalogy. Metabolic alkalosis. 65-The following conditions will present with cyanosis in the first week of life: 1. Skull radiograph shows 'pepperpot' skull 15 . Hypoplastic left heart syndrome 4. Aortic stenosis 2. Is a predominantly autoimmune syndrome. 5. 2.

Intravenous antibiotic 5. Intravenous fluid 3.PEDIATRICS EXAMINATION REVIEW 67-Hypochromic microcytic anaemia is seen in: 1. Folate deficiency 5. IL-1 causes pyrexia and induces protein synthesis in the liver 2. Inflammatory bowel disease 2. Can be used clinically to help treat infection 5. Granulomatous lesions 5. Normal levels of IgA 4. Sickle cell anemia 3. Lumbar puncture 16 . Her teacher at school has reported that she has numerous episodes throughout the day of abrupt ly staring into space midconversation for a few seconds with rapid return of awareness. The first management step would be : 1. Autoimmune disease 3. Abnormal features on MRI scanning of the brain 4. Throat swab 4. Susceptibility to recurrent parasitic infections 69-A 9 yr old girl is seen in clinic. IL-2 s the main cytokine produced by naïve T cells 72-15-year-old teenager presented with few haehorrha gic spots. Temp 39. Alpha thalassemia 2. Peak flow monitoring can be used in most children aged 5 years and above 71-Regarding cytokines:1. Inhaled glucocorticoids improve lung function in asthmatics 5. Provocation of the episodes by hyperventilation 3. Oxygen 2. agitation and confusion having been to school. Autoimmune hemolytic anemia 4. Which of the following features suggest complex partial rather than absence seizures as the cause of her problem : 1. The diagnosis of asthma involves blood tests 3. pulse 110 and BP 120/80. Interferon gamma causes increased expression of MHC class II molecules on antigen presenting cells 3. Imerslund syndrome 68-Which of the following is associated with common variable immunodeficiency? 1. A good response to anti-epileptic drugs 5. Reporting unusual smells prior to the episodes 2. Chemokines can induce the transmigration of T cells into inflamed tissues 4. A normal EEG 70-The following statements are reagrding asthma: 1. Leukotriene antagonists are a first line treatment in asthma 2. Treatment can be divided into those that prevent and those that relieve symptoms 4.

Decreased sensitivity to opioids 77-The following tumour markers are strongly associated with the tumours shown: 1. 5. Aortopulmonary window 2. IgA and IgE levels are often low 5.PEDIATRICS EXAMINATION REVIEW 73-The following congenital lesions are associated with cyanosis: 1. Protein C deficiency 5. CA-125 and pancreatic carcinoma. Total anomalous pulmonary venous drainage (TAPVD) 3. CEA and colorectal carcinoma. 75-In Wiskott -Aldrich syndrome:1. Intolerance to heat 3. 3. Tetralogy of Fallot 5. 4. 5. Factor V Leiden mutation 4. Behcets syndrome 2. Coarctation of the aorta 4. Platelets are of normal size. Alpha-FP and hepatocellular carcinoma. Myopathy 5. Beta-HCG and choriocarcinoma. Dry skin 4. The immunodeficiency is restricted to T cells only 2. There is an increased risk of malignancy 4. 2. Inheritance is autosomal recessive 76-The following are consistent with myxedema: 1. but are few in number 3. 2. Amyloidosis. Bradycardia 2. Metastatic cancer. Eisenmenger's syndrome 74-Causes of hypoadrenalism include: 1. 4. Polycythaemia 17 . CA-19. Tuberculosis.9 and ovarian carcinoma. Cushing's disease. 3. 78-Arterial thromboses are associated with: 1. Atrial fibrillation 3. Severe sepsis.

Treated with regular C1 inhibitor infusions 83-Regarding the meninges: 1. Subdural hematomas form a lentiform shape 3. Breast development stage 3 refers to breast buds 3. Danazol is used for treatment 81-Regarding Tanner stages of puberty: 1. Pia stimulation during surgery is very stimulating 5. Asymmetrical breast development is common in girls during puberty 5. Arachnoid folds project inwards to form the falx cerebri and falx cerebelli 4. Boys' genital development is described by 5 stages 2. Inherited in an autosomal recessive fashion 2. C4 levels tend to be low during an attack 5. Rightward shift of the heart 18 . May cause a rise in insulin release 5. Upper airway obstruction 4. A consequence of C2 kinin production 3. Attacks can be triggered by exercise 4. Menarche is occurring in increasingly younger girls over the past three decades 82-Hereditary angioedema is:1. Rickets 3. Can cause an increase in PEFR 2. It is due to deficiency of complement component C1 2. Has no effect on the FEV1 3. Exercise limitation 5. Pia is responsible for production of CSF 84-Pectus excavatum is associated with: 1. Marfan's syndrome 2. The dura is adherent to the bone 2. Pubic hair in the prepubertal state is described as Stage 0 4. Has no effect on hypoxic-pulmonary vasoconstriction 4.PEDIATRICS EXAMINATION REVIEW 79-Salbutamol: 1. It can present with abdominal pain and vomiting 3. Causes hypokalemia after prolonged use 80-Regarding hereditary angioede ma: 1. Sometimes treated with the anabolic steroid danazol 5. A cause of spontaneous life-threatening laryngeal oedema 4.

4. Neuromuscular drugs should have been stopped for at least twelve hours. Has a false negative result of 10 . It is recommended in all infants born to HIV infected mothers 2. The enzyme Type 1 5 alpha reductase is expressed in sebaceous gland ducts 88-The following criteria must exist before a diagnosis of brain death can be made: 1.20% when looking for renal scarring. IgG2 subclass deficiency may also be present with more severe infective complications 5. is associated with HLA-DR3 19 . 3. Intravenous Pentamidine is recommended 3. Recommended when CD4 count drops below 1000microlitre 5. 2. May present with autoimmune disease 4. 86-Regarding P.45% when looking for renal scarring. 4. Oral isotretinoin causes hyperlipidaemia 5. No eye movement when 20 mls cold water is perfused into the external auditory meatus. Interleukin 1 alpha promotes comedogenesis 4. dilated pupils unresponsive to light. 5.PEDIATRICS EXAMINATION REVIEW 85-Renal ultrasound: 1. Decreases calcium and increases phosphate reabsorption in kidney 5.50% when looking for VUR. 2.Carinii prophylaxis: 1. is very rare (incidence less than 1 in 10000) 3. Females with acne commonly have elevated blood androgens 2.25 dihyroxy vitamin D3 production in the kidney 4. Carinii infection warrants prophylaxis 4. Is a good means of detecting distal ureteric dilation. Is a reliable means of looking for vesico ureteric reflux (VUR). Increases calcium and decreases phosphate reabsorption in kidney 2. The overall effect is to increase calcium and phosphate level in the blood 90-Selective IgA deficiency: 1. Has a false positive rate of 25 . Is treated with iv sandoglobulin 2. No motor response fr om painful stimuli. Fixed. Previous P. Absent gag reflex with pharyngeal stimulation. 3. Increases 1. Increases osteoclastic activity 3. 5. Has a false negative result of 15 . Oral steroids are a recognised cause 3. 89-The following are true regarding parathyroid hormone actions: 1. Oral candidiasis in HIV patients warrants Prophylaxis 87-The following are true about acne: 1.

May be used safely in patients with suspected raised intra -cranial pressure 5. Is typical of Noonan syndrome 3. Morphine. Is associated with strokes in 25% of patients by the age of 45 years 4. 3. Acts on the distal tubule of the kidney to increase reabsorption of water 4. 5.PEDIATRICS EXAMINATION REVIEW 91-The following increase vasopressin secretion: 1. The superficial epigastric artery is a branch of this vessel 95-Coarctation of the aorta: 1. Secretion can be suppressed by morphine 5. Alcohol. In the lower third of the thigh. Runs as a single trunk from the inguinal ligament to the lower border of the popliteus 2. Is typical of Marfan’s Syndrome 2. Vomiting. Is a direct continuation of the internal iliac 3. 20 . Exercise. Is more emetogenic than thiopentone 3. Is contra-indicated in reversible airways obstruction 2. Secretion is increased by pain . Has anti-sialogogue properties 4. 5. especially in the neonatal period. Is a potent respiratory depressant 93-Sickle cell disease : 1. Is caused by a mutation on chromosome 16 2. Will give a positive sickle test result at birth 3. Radiofemoral delay is the diagnostic sign 96-Anti-diuretic hormone (ADH): 1. Has a half-life in the plasma of approximately 24 hours 3. lies in the adductor canal (Hunter’s canal) 5. 4. In the upper third of the thigh the femoral artery is contained in the femoral triangle (Scarpa’s triangle) 4. Haemorrhage. Is a decapeptide 2. Renal failure may arise. Is associated with notching of the anterior ribs 4. 92-Regarding ketamine: 1. Is an indication for autologous haemopoietic stem cell transplant 5. 2. Can be co-inherited with alpha or beta -thalassaemia 94-The femoral artery: 1.

Sleep terrors last at least twenty minutes 4. Nightmares are associated with intense vocalisation 102-Nitric Oxide (NO): 1. Sleep terrors occur in non -REM sleep 2. Neurofibromatosis type I 2. Sleep terrors are associated with intense vocalisation 5. 2. Actinic keratosis 5. 21 . Hereditary spherocytosis 3. G6PD deficiency 99-The following skin conditions are by definition associated with epithelial dysplasia: 1. Reduction in pulmonary flow can cause a decrease in surfactant production 4.PEDIATRICS EXAMINATION REVIEW 97-In recurrent abdominal pain in children. Is the same substance as EDHF. pain localising close to the umbilicus 3. Increases cAMP. Xeroderma pigmentosum 2. Galactosaemia 5. Synthesis is stimulated by glucocorticoids 101-Regarding sleep disturbance: 1. 5. Synthesis is inhibited by thyroxine 5. 4. Bowen’s disease of skin 3. 3. presentation at age 10 2. Operates by inhibiting PDE’s. Congenital adrenal hyperplasia 4. the following suggest an underlying organic cause: 1. Is produced by type 1 alveolar cells 3. Nightmares are associated with appre ciable body movements 3. Is critical in increasing endothe lin (ET-1) levels. Basal cell carcinoma 4. family history of migraine 4. Is partly recycled by endocytosis into the synthesizing cell 2. Seborrhoeic keratosis 100-Pulmonary surfactant 1. Is important in the maintenance of pulmonary vascular flow. vomiting 98-The following are inherited in an autosomal dominant manner 1. pain during daytime only 5.

PEDIATRICS EXAMINATION REVIEW

103-Factors associated with poor wound healing include:
1. Vitamin A deficiency 2. Vitamin B1 deficiency 3. Vitamin C deficiency 4. Folate deficiency 5. Zinc deficiency

104-The following are true of COPD exacerbations: 1. All patients should be given antibiotics 2. Are associated both with rhinovirus and chlamydia pneumoniae infection 3. There is a positive correlation in mortality and the number of smoking pack years 4. pH less than 7.35 is an indication for antibiotics according to the BTS guidelines 5. Moraxhella catarrhalis is the most freque ntly implicated organism in exacerbation

105-The following statements are correct regarding the cardiac anatomy:
1. The left atrium is larger in size as compared to the right atrium. 2. Right coronary artery is dominant in over three -fourth of cases. 3. There is one pulmonary vein from each lung that enters the posterior wall of the left atrium. 4. The AV node is supplied by the left anterior descending artery in most of the cases. 5. Most of the venous drainage of the heart occurs via the the Basian system of veins.

106-Mitral valve prolapse is associated with: 1. Cyanosis 2. Bacterial endocarditis 3. Chest pain 4. A systolic 'click' on auscultation 5. Foetal arrythmias

107-Normal arterial pH is :
1. Equal to [H+] of 34-46 nmoles/L 2. Lower than intracellular pH 3. Not essential to maintain ion distribution 4. Measured rather than calculated in a pH probe 5. Decreased in pregnancy

108-A 16 year old girl is admitted with progressive weight loss for whi ch no organic cause can be found. The following factors would be against a diagnosis of anorexia nervosa: 1. A history of laxative abuse 2. The delusion that someone is trying to control her eating patterns 3. An excess of physical exercise 4. Self-induced vomiting 5. Low plasma potassium

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PEDIATRICS EXAMINATION REVIEW

109-Defective Fas-FasL interaction leads to impairment of : 1. CTL-mediated lysis 2. Helper T-lymphocyte activation 3. NK cell killing 4. Oxidative burst within activated macroph ages 5. Recruitment of neutrophils

110-The following poisons are matched to the appropriate therapy: 1. Carbon monoxide - hyperbaric oxygen 2. Organophosphates - atropine and pralidoxime 3. Beta-blocker - phentolamine 4. Methanol - ethanol 5. Tricyclic antidepressants - phenytoin

111-The following are true:
1. The uptake of iodide into the thyroid follicular cell is by active transport 2. T4 has a half-life of 24 hours 3. About 99% of circulating T4 is protein boun d 4. T3 is roughly 10 times more potent than T4 5. All steps in thyroid hormone synthesis are stimulated by thyroid peroxidase

112-Restriction fragment length polymorphisms (RFLPs): 1. Are found every 100 bases 2. Are useful in diagnosis of multifactorial diseases 3. Are useful in linked-gene diseases 4. Are generated by digestion of DNA with bacterial enzymes 5. Are detected by Northern blotting

113-Which of the following statements are true:
1. Data may be described as qualitative or quantitative 2. Discrete data may include number of children 3. Discrete data may include serum glucose values 4. Ordinal data does not include tumour staging 5. Nominal data may include blood group type

114-In an athymic child:1. Macrophage function is impaired 2. The development of gamma/ delta -T-lymphocytes is unaffected 3. The production of antibody is unaffected 4. The function of helper T-lymphocytes (Th) is affected while cytotoxic T -lymphocytes (Tc) activity is only moderately impaired 5. Lymphadenopathy is common because of the over -expansion of B-lymphocytes

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PEDIATRICS EXAMINATION REVIEW

115-The following statements are true about the mitral valve
1. It is bicuspid 2. It lies posterior to the sternum at the level of the fourth costal cartilage 3. The associated papillary muscles are smaller than those of the tricuspid valve 4. Its' opening is associated with the first heart sound 5. It can be regurgitant even if structurally normal

116-Causes of vesicular rash in children include: 1. Roseola Infantum 2. Dermatitis herpetiformis 3. Hand Foot and Mouth disease 4. Kawasaki disease 5. Molluscum contagiosum

117-The following are causes of lymphadenopathy:
1. Rubella 2. Amyloidosis 3. SLE 4. Rheumatoid arthritis 5. Thyrotoxicosis

118-The following statements are true for the Renin Angiotensin System: 1. Renin converts Angiotensin I to Angiotensin II in the kidney 2. Angiotensin II promotes renal arteriolar dilatation 3. Angiotensin II inhibits aldosterone secretion by the adrenal glands 4. Angiotensin II receptors are blocked by spironolactone 5. Losartan inhibits formation of Angiotensin II

119-Which of the following are common symptoms associated with raised intracranial pressure in children?
1. Morning headache 2. Abdominal pain 3. Double vision 4. Ataxia 5. Seizure

120-The following is true regarding endotoxin: 1. It is a protein 2. It is heat-stable 3. It is produced by some Gram-positive bacteria 4. All endotoxin has the same physiological effect 5. It is secreted by bacteria during active infection

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PEDIATRICS EXAMINATION REVIEW

121-The following are zoonoses:
1. Legionnaires’ disease 2. Hydatid disease 3. Lyme disease 4. Poliomyelitis 5. Salmonellosis

122-Regarding Parvovirus infections in children: 1. It is the causative agent for exanthem subitum 2. Arthritis is a very common manifestation in children 3. Chronic anemia can be a manifestation in some children 4. Infection in pregnancy can result in fetal anemia 5. Aplastic crises due to parvovirus infection in sickle cell disease usually requires bone marrow transplant

123-Definition of status epilepticus:
1. Two or more fits within 30 minutes 2. Unresponsive patients who have been incontinent for 60 minutes 3. Mouth twitching lasting 60 minutes with reduced GCS 4. Tonic-clonic seizure lasting 30 minutes with incontinence 5. Rapidly repeating tonic -clonic seizure with not returning to full c onsiousness

124-Regarding Gross Motor Development, a normal child would be expected to: 1. Roll over by 2 months 2. Ride a tricycle at 2 years 3. Kick a ball at 2 years 4. Cruise by 1 year 5. Not run until 2 years

125-The functional consequences of moderately severe mitral stenosis include:
1. Reduced lung compliance. 2. Hypercapnia. 3. Reduced glomerular filtration rate. 4. Raised left atrial mean pressure. 5. Reduced pulmonary vascular resistance.

126-Evaluate the truth of the following descriptions of cytokines and their functional activities:1. IL-3 : growth and differentiation of bone-marrow stem cells 2. IL-2 : activation induced cell death 3. IL-10: activation of B-cells 4. IL-6: induction of antibody secretion by plasma cells 5. IL-12: induction of IFN-gamma secretion by activated T-cells

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Measured rather than calculated in a pH probe. CXR commonly shows hyperinflation. Lower than intracellular pH. functional minimum urine output/day in 70kg male is 1400mls 128-In children with renal failure 1. 4. normal Na content is <20mmol/L 3. Not essential to maintain ion distribution. Causes an increase in chest wall compliance 3. dialysis is indicated with rapidly rising sodium 2.PEDIATRICS EXAMINATION REVIEW 127-Urine 1. Equal to [H+] of 34 . Is a glycoprotein 4. 5. 5. Secretin inhibits pancreatic bicarbonate secretion 5. 2. RSV immune globulin is recommended in infants with cyanotic congenital heart disease to prevent severe RSV infection. 3. microangiopathic haemolytic anaemia is characteristic 5.5 2. 4. Ribavarin is curative. normal pH is >5. Decreased in pregnancy. 3. Decreases the surface tension within an alveolus 2. 26 . Lymphopenia is common. Pancreatic polypeptide inhibits pancreatic secretion 132-Normal arterial pH is: 1. Grade V vesico-ureteric reflux spontaneously resolves in only 10% 129-Lung surfactant 1. Glucagons increases GI motility 4. the following are true : 1. normal osmolality is 300 -600 mosmol/kg 4. In haemolytic uraemic syndrome. Protein intake should be restricted in chronic renal failure.46 nmoles/L. Anaemia of chronic renal failure is due to low plasma ferritin levels 3. Gastric distension increased motility 3. Appears only after the 1st week of life 130-With regards to bronchiolitis. 4. protein content of 500mg/day is within normal range 5. It is linked to maternal smoking. 131-The following concerning gastrointestinal hormones are true 1. In acute renal failure. Maintains the same surface tension for different sized alveoli 5. 2. Somatostatin reduces gastric motility 2.

Most of the alveoli in the adult lung are present at birth 4. 2. The vagus nerve is responsible for normal bronchial tone 2. 3. Gastric carcinoma frequently metastasises to the brain. Urinary lead concentration is the most useful indicator of exposure 2. 5. Diagnosis can be made by Haemoglobin electrophoresis 4. Chlamydia trachomatis 3. Neisseria gonorrhoeae 134-The following statements are true: 1. Is associated with basophilic stippling in white cells 138-Benign rolandic epilepsy :1. Anaemic patients should receive regular iron supplementation 5. Haemodialyisis effectively eliminates lead 4. In asymptomatic children with increased lead levels DMSA therapy is recommended 3. Can be successfully treated with carbamazepine 5. The reticulocyte count is low 3. Beta thalassaemia major presents at birth with profound anaemia 2. Splenectomy is contraindicated due to extramedullary haemopoiesis 136-The following statements concerning CNS tumours are true: 1. Adenovirus 4. 4. 137-Regarding lead poisoning:1. The major component of surfactant is dipalmitoyl lecithin 135. Has a typical EEG with a 3 per second spike and wave pattern 27 . Glioblastoma multiforme is a benign neoplasm. Haemophilus influenzae 5. Epstein Barr virus 2. Is a common form of partial epilepsy 2. Astrocytomas arise from the neuroglia. Involves seizures that often occur at school 4. The surface markings of the right middle lobe approximate to the axilla 5. Cerebral metastases comprise around 70% of all brain tumours.In beta thalassaemia: 1. Meningioma is usually benign.PEDIATRICS EXAMINATION REVIEW 133-The following organisms cause conjunctivitis: 1. The half-life of the deep bone compartment is months 5. There are 18 bronchopulmonary segments 3. Has a poor prognosis 3.

Churg-Strauss 3.PEDIATRICS EXAMINATION REVIEW 139-Which of the following biological responses or consequences is mediated b y the release of tumour necrosis factor (TNF) –alpha? 1. The lymphoid function is carried ou t in the white pulp. 5. Absence of sweating in the limb 143-Autism :1. Following splenectomy. 4. 141-Which of the following would cause an increase in the carbon monoxide transfer factor? 1. Can be treated successfully with psychotherapy 2. 2. 3. He is found to a spinal schwannoma. Is related to Rett syndrome 144-With regards the spleen: 1. The superior intercostal ar tery. Exercise 5. low dose penicillin & pneumovax are recommended. 2. Which of the following is likely to result from this? 1. Induction of killing by cytotoxic T lymphocytes (CTL) 140-The neck of the 1st rib is related to: 1. Goodpasture’s syndrome 4. 4. temperature and preservation of light touch 4. Scalenus anterior. Resetting of the temperature threshold in the hypothalamus resulting in pyrexia 5. Anaemia 142-A patient develops low back pain which is progressive. Stellate ganglion. Spinothalamic tract degeneration above the section level 5. 28 . 3. it is largely the kupffer cells in the liver that filter micro -organisms. Extra-pulmonary restriction 2. Production of acute phase proteins by hepatocytes 2. Loss of motor function 2. Disseminated intravascular coagulation in systemic Gram -negative bacterial infection 4. Loss of pain. Mobilisation of B-cells from the bone marrow 3. Basophilic stippling of neutrophils is seen with asplenia. Is related to Fragile X syndrome 4. The spleen is involved in the destruction of enc apsulated organisms. but division of the posterior spinal root occurs. Loss of tendon reflex 3. Is sometimes related to poor parenting 3. Is related to Sandifer Syndrome 5. Following splenectomy. 5. It is resected. The thoracic duct on the right. Phrenic nerve.

2. Should be treated aggressively in the early stages of the disease 4. ASD’s account for 40% of all CHD 3. Should recognise his/her name 2.PEDIATRICS EXAMINATION REVIEW 145-Chronic lymphocytic leukaemia :1. 29 . 80% of children with Down’s Syndrome have CHD 5. Ondansetron 149-A normally developing 18 -month-old child: 1. VSD’s account for 70% of all CHD. Approx. Patients have defective T-cell dependent antibody responses and defective cell -mediated immunity 2. Is asymptomatic at diagnosis in less than 10% of pati ents 3. Patients are susceptible to infection with bacte ria and viruses 3. Leukotriene inhibitors are associated with the development of Churg -Strauss . Approx. Is associated with splenomegaly at diagnosis in about 50% of patients 146-In asthma:1. X-linked SCID is caused by a defect in the IL -2 receptor 4. Ganglion blockers 5. Blocking interleukin-5 maybe therapeutic 4. Is associated with smear or smudge cells in the peripheral blood 2. The eosinophils to the lung are recruited from the bone marrow 2. 13% of neonates with chromosomal abnormalities have associated CHD 4. Progesterone 3. SCID can be due to an autosomally inherited defect in DNA repair analogous to the genetic defect in the SCID mouse strain 148-Lower esophageal sphincter tone is decreased by: 1. Should be able to copy a vertical line 150-The following statements concerning congenital heart disease (CHD) are true: 1. Should imitate observed actions 5. Steroids are contra-indicated if the patient is a diabetic 3. Has a median survival of less than 5 years 5. 5. Approx. Plays in parallel with his/her peers 4. Suxamethonium 4. Cisapride 2. ADA (adenosine deaminase) and PNP (purine nucleotide phosphorylase) deficiency cause accumulation of nucleotide metabolites that are toxic to developing T -cells resulting in T-cell and B-cell deficiencies and a SCID phenotype 5. 147-With regards severe combined immunodeficiency: 1. 40% of patients with Turner’s Syndrome have coarctation and -or aortic stenosis. Can easily distinguish primary colours by name 3. Bronchial hyper-responsiveness (BHR) does not have to be present.

Perianal skin tags 5. A lesion of the left occipital lobe will result in a right homonymous hemianop ia 3. Ipsilateral ptosis 2. Metabolic alkalosis is the reduc tion in H+ concentration by any means other than decreased PCO2.With regard to the visual pathways: 1. Levator veli palatini 4. Contralateral mydriasis 3. Loss of sweating over the ipsilateral face 4. The pupillary light reflex is preserved in lesions of the optic nerve 4. Purpuric rash suggestive of Henoch-Schonlein purpura (HSP) 2. Increased central chemoreceptor firing rates decrease ventilation rates. Ventilation is reflexly depressed by metabolic alkalosis. 155. Malabsorption 2. Metabolic alkalosis can be induced by vomiting from the stomach. Gestational diabetes 155-Metabolic influences on pH 2: Which of the following statements are true? 1. Palatoglossus 3. 2. Hyperventilation induced by metabolic alka losis returns arterial pH to the normal pre -set points. 5. 4. Acromegaly 3. Partial gastrectomy 4. 3. A pituitary adenoma may cause a bitemporal hemianopia 30 . Webbed neck and low hairline 4. Haematochezia due to peptic ulceration 152-The muscles of the soft palate are: 1.PEDIATRICS EXAMINATION REVIEW 151-The following are clues to the cause of an upper gastrointestinal (GI) bleed: 1. Tensor veli palatini 2. Lesions of the optic nerve cause an enlargement of the blind spot 5. A lesion of the optic chiasma will cause a homonymous hemianopia 2. Hyoglossus 5. Exophthalmos 154-An abnormal response to oral glucose loading is seen in: 1. Wilson's disease 5. Palatopharyngeus 153-Horner’s syndrome invariably produces :1. Hypopigmented spots on the lips suggestive of Peutz -Jeghers syndrome 3. Heterochromia 5.

GFR can be measured using a thermodilution technique 159-In enuresis :1. Consciousness is altered 3. GFR can be measured using Doppler ultrasound 5. Methemoglobinemia 3. Fetal Hb 2.2 3. Kawasaki disease 5. Idiopathic thrombocytopenia purpura 4. Carcinoid syndrome 161-Regarding complex partial epilepsy: 1. Hyperparathyroidism 3. 5% of 5 year old children are affected 3. EEG changes disappear in sleep 5. Urine dipstick is a useful investigation 4. Conditioning is usually successful 160-Endocrine disorders that result in diarrhoea include: 1. Hyperventilation often provokes a complex partial seizure 4. Cushing’s syndrome 2. Primary hyperaldosteronism 5. IgA deficiency 3.HCT) 4. Renal blood flow = plasma flow/(1 . Absence seizures and generalised tonic -clonic seizures may occur 2. The filtration fraction is the ratio of GFR to renal blood flow 2. Normal filtration fraction is 0. Increased 2.1-0. In a 3 year old investigations must be carried out 2. Thyrotoxicosis 4. Increased lactic acid production 4. Metachromatic leukodystrophy 158-In the kidney 1. Ethosuximide is an affective treatment 31 .PEDIATRICS EXAMINATION REVIEW 156-The O2-Hb dissociation curve is displaced to the right in: 1. First line treatment is imipramine 5. Guillain-Barre syndrome 2.3 DPG 157-Intravenous immunoglobulin (IVIG) is used in the treatment of: 1. Chronic hypoxia due to cyanotic conditions 5.

Results in wide splitting of the second heart sound 3. Galactosemia 3. Diagnosis is usually made by bone biopsy 163-Ventricular septal defects: 1. Has an oral bioavailability of 50% 4. Are secreted from the zona fasciculata within the adrenal cortex. 2. Prolongs the duration of action of suxamethonium 3. 4. There is an increased incidence of osteosarcoma 2.PEDIATRICS EXAMINATION REVIEW 162-In osteogenesis imperfecta 1. Septicemia 166-The following statements are true: 1. Hyperthyroidism 4. Cause retention of sodium. Has been used in the treatment of SVT 2. Relapsing polychondritis 5. Pamidronate is a recognised treatment in children with this condition 4. A newborn baby from 32 weeks gestation will turn tow ards a light 167-Glucocorticoids: 1. Cardiac bypass is not necessary for surgical correction 164-Neostigmine: 1. An extensor Babinski response is normal in the first year 5. A moro reflex with the fists remaining clenched is normal. Is metabolized by plasma esterases 5. 32 . Hearing abnormalities are common 5. Can present with heart failure in the neonatal period 2. The plantar grasp persists longer than the palmar grasp 3. 5. 3. Blue sclera is diagnostic 3. Reduce lymphocyte counts. Deep tendon reflexes are difficult to elicit at birth 4. May regress by incorporation of the tricuspid leaflet tissue 4. Muscular dystrophy 2. Molecules consist of 21 carbon atoms. Crosses the placenta 165-Recognized associations of systemic conditions and cataracts include: 1. 2. Inhibit gluconeogenesis. Tricuspid atresia is a recognised association 5.

4. 5. Is related to the common bile duct posteriorly 33 . Causes a rise in metabolic rate 5. Thyroid peroxidase catalyses the formation of thyroxine. 170-The following diseases are inherited in an autosomal recess ive manner:1. T4 is released bound to thryroglobulin. Is a neurotransmitter at the pre-ganglionic sympathetic nervous syst em 2. 2. Behavioural therapy is never useful 2. Is entirely retroperitoneal 2. Parenting techniques can be taught 5. Is crossed by the attachment of the transverse mesocolon 4. Is crossed by the left renal vein 5. Congenital adrenal hyperplasia 5. Primary ciliary dyskinesia 3. 3. Poor appetite is a common side affect of methylphenidate (Ritalin) 169-Concerning the synthesis of thyroid hormones: 1. Iodine is taken up passively by the thyroid gland. Pyloric stenosis 171-Noradrenaline 1.PEDIATRICS EXAMINATION REVIEW 168-In the treatment of attention deficit hyperactivity disorder : 1. Batten's disease 4. TRH is released by the anterior pituitary. Functional closure of the foramen ovale in the first 24 hours 2. Methylphenidate (Ritalin) should not be given late r than 4pm 4. May cause a reflex bradycardia when given by infusion 4. Is supplied by branches of th e coeliac artery alone 3. T4 is released in greater quantities than T3. The ductus arteriosus closes in response to decreased oxygen concentrations 5. Decrease in pulmonary artery resistance following closure of the ductus arteriosus 4. Blood flow in the ductus arteriosus continues from right to left until its closure 3. The umbilical artery is a branch of the common iliac artery 173-The pancreas: 1. Pharmacological intervention includes the use of stimulant drugs such as amphetamines 3. Insulin dependant diabetes 2. It acts predominantly via the beta adrenoceptor 172-In a healthy baby the transition from fetal to neon atal circulation involves:1. Is produced by the adrenal cortex 3.

2. The defect in Chediak-Higashi syndrome is in T-cell activation 4. Is seen in Kartagener's syndrome 2. Intra uterine growth retardation 5. Maxillary hyperplasia 3. Is usually asymptomatic 3.PEDIATRICS EXAMINATION REVIEW 174-IgA deficiency:1. Deficiency in glucose-6-phosphate dehydrogenase results in impaired motility of leukocytes 5. The incidence of lymphoid malignancy is increased in ataxia telangiectasia 3. Hepatitis C. May delay diagnosis of coeliac disease 4. 4. Hepatitis B. Affects both serum and secretory IgA levels 175-Abnormalities associated with foetal alcohol syndrome include: 1. Has bilateral cortical representation. Moya moya disease 178-The following statement(s) concerning inherited immunodeficiency syndromes are true: 1. Tuberculosis. Malaria. Nephrotic syndrome 2. The commonest inherited form of severe combined immune deficiency is x -linked 2. 4. 3. HIV. Systemic lupus erythematosis 4. Receives taste fibres from the palate. 5. Has a sensory supply in the ear canal. Activated protein C resistence 3. When lesioned below the level of the stylomastoid foramen will cause hyperacusis. Short palpebral fissures 2. Haemoglobin SS 5. Receives taste fibres from the anterior 2/3rd of the tongue via the greater petrosal nerve. Hyperactivity in childhood 4. 5. 2. Spontaneous recovery is a recognised feature 5. 177-Strokes in childhood are associated with :1. 34 . Hypotonia 176-The seventh cranial nerve: 1. 3. The molecular defect in X -linked hyper IgM syndrome is in the expression of CD40 on B -cells 179-The following diseases may be transmitted by needlestick injury: 1.

4. Does not need to be investigated in a girl 5. Maintains a uni-directional flow of lymph towards the thoracic ducts 3. Sugar 183-Co-administration of the followin g pairs of drugs results in reduced absorption of the latter:1. Sucralfate and phenytoin 4. Is more common around the New Forest 181-The lymphatic system: 1. 3. Is a valveless system 2. May be diagnosed on urine culture 3.PEDIATRICS EXAMINATION REVIEW 180-Leptospirosis: 1. Has a total lymph drainage of 6-8 litres of fluid/24 hours from interstitial fluid 5. Antacids and prednisolone 2. Contains lymph draining from the liver and intestine with a protein concentrati on of approximately 40 -60 g/l 182-The following have a much lower concentration in the cerebrospinal fluid (CSF) than in the cerebral capillary blood. Spinal cord imaging may be indicated 3. Most recover within two weeks without treatment 2. Leads to increased levels of plasma angiotensinogen 2. 2. The thoracic ducts drain into the venous circulation at the junction of the sub -clavian veins and internal jugular veins 4. Leads to sodium retention. Cholestyramine and warfarin 3. Osmolarity 4. Is due to atrophy of zona glomerulosa. 1. 5. Is treated with tetracycline 4. Serum creatine kinase should be measured 4. Causes muscle weakness. Is more common in females. Amitriptyline and digoxin 5. Metoclopramide and paracetamol 184-Toe-walking in an 18 month old child :1. 35 . Is always abnormal 2. Na+ 3. Protein 5. May be a sign of cerebral palsy 185-Conn's syndrome: 1. K+ 2. May be complicated by thrombotic thrombocytopenic purpurea 5.

Goldenhar 36 . The isobestic point will vary with hemoglobin concentration 3. Infective endocarditis 3. Is associated with HLA B8 5. The clark electrode uses a silver/silver chloride cath ode 5. Is associated with vitamin C deficiency 2. Vacterl association. Plasmodium malariae infection 190-Regarding oxygen measurement: 1. 189. CHARG association. Release cytokines wich affect the adaptive immune system 4. Vision is commonly decreased. Initiate firm attachment by binding to ICAM on the endothelium 188-With regards to aniridia: 1. 5. Water vapor will cause inaccurate readings from a paramagnetic analyzer 191-The following syndromes/ associations are associated with congenital heart disease. It is usually bilateral. Polyarteritis nodosa 5. Is more common in men 4. 3. Two thirds of the cases are sporadic. Is associated with Jaccoud's arthropathy 3. The readings from pulse oximeters are affected by anemia 4. Wilm's tumour is associated with familial aniridia.PEDIATRICS EXAMINATION REVIEW 186-Haemochromatosis:1. holt-Oram syndrome. Is associated with a gene on chromosome 13 187-Phagocytic Cells :1. 1. 2. Are important for the resolution of inflammation 5. Have receptors that recognise bacterial cell wall constit uents 2. 4. 4. Systemic lupus 4.Immune complex deposition in the kidney occurs in: 1. It is associated with partial deletion of long arm of chromosome 11. 3. Goodpasture's disease 2. Require clonal expansion to mount an effective response 3. Red light absorbs more oxyhemoglobin than deoxyhemoglobin 2. 5. Septo-optic dysplasia. 2.

Chronic mucocutaneous candidiasis. 5. Constriction of the pupil. All patients should be on inhaled steroids. 196-Complete transaction of the oculomotor nerve results in : 1. 4. 3. Convergent squint. Hypothyroidism. 4. 4. EMG shows decremental response to repetitive motor nerve stimulation. Patient education is emphasized. Left handedness is more common in exprem babies without cerebra l palsy. 2. 3. 5. Cystic fibrosis. Haemolytic disease. 2. 4. Chronic granulomatous disease. High dose regular short acting bronchodilators should be tried before inhaled steroids. 2. 4. The neonatal form is always due to maternal antibodies. Thyroid dysfunction is a recognised association. 5. Di George syndrome. 193.PEDIATRICS EXAMINATION REVIEW 192. The diagnostic test is subcutaneous endophonium. Alpha-1-antitrypsin deficiency. 3. Cytomegalovirus infection. Leukotrene inhibitors are recommended in step 4. Wiskott-Aldrich syndrome. 5. Either long acting beta-2 agonists (LABAs) and low dose steroid or high dose inhaled steroid can be used to the same clinical effect.Regarding child development: 1. conjugated hyperbilirubinaemia may be caused by: 1. 3. Sense of taste is present from birth. 4. 37 . 5. 195-Persistant. 2. Left handed children are more likely to have un intentional injuries. Loss of accommodation and light reflex. The disease can be confined to ocular muscles. 3. 2. 5. 3. 2. 194. 197-The following involve deficiencies in T -cell immunity: 1. Ptosis. Bruton’s agammaglobulinaemia. Diplopia.Regarding myasthenia gravis in childhood: 1.The following statements regrd the treatment of asthma: 1. 25% of children are left handed. Prevalence rate of thumb-sucking in children are universal across races.

38 . Increased red cell volume and MCH. 199-The following maternal conditions are known to cause adverse effects on the neonate: 1. 5. Night terrors occur in the stages of deep sleep. 5. 4. Alkalosis. 2. Chronic myeloid leukaemia. 4. 200-en uptake by haemoglobin is characterized by: 1. Increased 2-3 DPG. Diabetes insipidus. 5. Sleep walking occurs during REM sleep. 4. 2. SLE. Sleep disorders are never treated with drugs. Raised temperature. Hyperthyroidism. 3. 2. Breath holding attacks cause cyanosis. Toxoplasmosis. 3. Breath holding attacks should be investigated with CT scan. 3. 1.PEDIATRICS EXAMINATION REVIEW 198-In young children. High altitude.

PEDIATRICS EXAMINATION REVIEW Answer &Teaching Notes for Questions (PART 1) 39 .

HBsAg is the first serological marker to appear 4. * Anti-HBs appears last (12 wks). The presence of HbsAg indicates acute infection only 5. Incubation of virus: 6 weeks – 6 months. Lead to excess stimulation of T cell proliferation and interleukin -2 production 4. 40 . vomiting. MARKERS AB: * Anti-HBc (IgM) .PEDIATRICS EXAMINATION REVIEW 1.The following statements are true regarding superantigens: 1. excess stimulation of T cell proliferation occurs leading to abnormally high levels of IL-2. fever and malaise. Lead to stimulation of production of TNFa and other cytokines Teaching Notes for Question 2 Theme: Superantigens Superantigens are an unusual type of bacterial toxin comprising of proteins which exert their effect by forming a bridge between the MHC complex of antigen -presenting cells and T cell receptors. 10–15% prevalence in Africa/Far East. but persists in chronic infection (patient then becomes a carrier). HBV only replicates in the liver 3. Indicates continued viral replication. until 12 wks.Regarding hepatitis B virus (HBV): 1. HBV is an RNA virus 2. * HbeAg appears at 6 wks and disappears at 10 wks as Anti -HBe rises ('seroconversion'). indicates immunity. It is possible that they may also have a role in the pathogenesis and clinical features of Kawasaki disease. indicates reduced infectivity. if found. MARKERS AG: * the first screening Ix is HBsAg. 2. a full profile is requested. They bind to B cell receptors 3. It is spread by blood Tx/IVDA/tatooists/sexually/vertically (most important worldwide).First to appear at approximately 8 wks. Most sensitive test of continued viral replication is PCR for HBV DNA. Persistence of HbeAg indicates increased severity and infectivity. They are very strongly antigenic bacterial polysaccharides 2. across Europe HBV and HCV cause approximately 50% hepatocellular carcinomas. * Anti-HBe appears next (10 wks). Because the binding to T cell receptors is indiscriminate. with symptoms of nausea. particularly toxic shock syndrome and staphylococcal food-borne disease. The persistence of HbeAg indicates infectivity Teaching Notes for Question 1 Theme: Hepatitis B HBV is a double stranded DNA virus (hepadenovirus) and only replicates in the liver. They have a role in Gram-positive septic shock. HbsAg appears at approximately 6–10 wks indicating acute infection and then vanishes if virus has cleared. They are responsible for gram-negative toxic shock 5.

g. bleomycin. Right-sided aortic arch. 3. cerebral metabolism. cyclophosphamide. sulfasalzine.The following are clinical associations of Fallot’s tetralogy: 1. temp. but not pulmonary fibrosis 4. Digital clubbing. 1. The CBF shows an exponential increase in the CBF at PaO2 levels of below 5. pericardial and subendocardial fibrosis. Penicillamine 4. 5. this gradually normalizes within 72 hours despite the low PaCO2. Studies have shown that although there is the initial cerebral vasoconstriction with the low PaCO2. Teaching Notes for Question 5 41 . Teaching Notes for Question 4 Theme: Cerebral blood flow The CBF is affected by PaO2. 4. and cytotoxics (e. 4. pleural. 4.5 Kpa but is fairly constant at levels above that. MAP. busulphan. Sulfasalazine 2.PEDIATRICS EXAMINATION REVIEW 3. 2. Chlorpromazine 3. ICP. Shows a right shift in the autoreg ulation curve in patients with chronic hypertension. 5. azathioprine. Is increased in an acutely compensated metabolic acidosis. chlorambucil. As it is acute there will be no change in the CSF pH. nitrofurantoin. Is decreased at 72 hours in a normal person who is made to hyperventilate for 72 hours. and methotrexate). Aortic ejection click. 2. Cerebral abscess.Causes of pulmonary fibrosis include:1. 2. Shows a linear increase with PaO2. NB: methysergide causes retroperitoneal. which will decrease the PaCO2. 5. A compensated metabolic acidosis is achieved via hyperventilation. Single second heart sound. Every decrease in body temperature of 1 degree celcius will decrease the cerebral metabolism by 7%. neurogenic factors and drugs. 3.Cerebral blood flow: 1. 5. Nitrofurantoin Teaching Notes for Question 3 Theme: Pulmonary fibrosis Causes of pulmonary fibrosis include amiodarone. PaCO2. melphalan. bleomycin. Methysergide 5. Is unaffected by temperature.

is caused by the obstruction of right ventricular outflow. 4. The first heart sound is normal. Patients with tetralogy of Fallot have cyanosis and digital clubbing. followed by worsening cyanosis and. 2. The genetic defect in X-linked agammaglobulinaemia (XLA) is in Bruton’s tyrosine kinase (Btk) Teaching Notes for Question 7 Theme: Hypogammaglobulinaemia In nephrotic syndrome. In childhood. with an upturned right ventricular apex and a concave main pulmonary arterial segment. 3. The heart is classically boot-shaped. a systolic thrill (caused by turbulent flow across the right ventricular outflow tract) is palpable. and endocarditis. a soft. bradycardia and rarely hyperpyrexia. short murmur suggests that severe obstruction is present. abnormalities of haemostasis. Selective IgA deficiency is the commonest inherited form 4. In patients over 40 years of age. Allows improved intra-abdominal access. and lung markings are diminished. A right-sided aortic arch may be present. Adults with tetralogy of Fallot have dyspnoea and limited tolerance of exercise. the magnitude of which is proportional to the severity of the desaturation. Is not necessary in ventilated patients. audible along the left sternal border. In some patients. Side effects include histamine release. In common variable immunodeficiency. They may have complications of chronic cyanosis. Such spells do not occur in adolescents or adults. there is an increased incidence of lymphoma 3. which of the followings are true? 1. hyperviscosity. 6 percent at 30 years. 6. Side effects include histamine release. the susceptibility to pneumococcal infection is multifactorial and includes decreased immunoglobulin levels. characterised by tachypnoea and hyperpnoea. the severity of which is determined by the degree of obstruction of the right ventricular outflow tract. and 3 percent at 40 years.vecuronium. Block acetyl-choline receptors on muscle end plates. On radiography.pancuronium) are longer lasting. 5. The electrocardiogram shows right-axis deviation and right ventricular hypertrophy. the loss of immunoglobulin in urine is the only cause of predisposition to infection 2. in some cases. a chest radiograph is mandatory to exclude thymoma 5. 11 percent at 20 years. 40 percent at 3 years. and even death. most patients die in childhood: the rate of survival is 66 percent at 1 year of age. since its pulmonary component is inaudible. overriding aorta) may be heard. Without surgical intervention. seizures. including erythrocytosis. but the second heart sound is single. Arterial oxygen desaturation is evident. A systolic ejection murmur.Muscle relaxation during surgery: 1. loss of consciousness. the size of the heart is normal or small. 7-In hypogammaglobulinaemia. In nephrotic syndrome. Non depolarising agents(atracurium. cerebrovascular accidents. An aortic ejection click (due to a dilated. Is maintained using a depolarising agent such as suxamethonium. cerebral abscesses or stroke. Teaching Notes for Question 6 Theme: muscle relaxants Depolarisng agents(suxamethonium) are short acting. such patients may have sudden hypoxic spells. as is compensatory erythrocytosis. The intensity and duration of the murmur are inversely related to the severity of the obstruction of right ventricular outflow. The peripheral pulses are normal.PEDIATRICS EXAMINATION REVIEW Theme: Fallot’s tetralogy Most patients with tetralogy of Fallot have cya nosis from birth or beginning in the first year of life. A right ventricular lift or tap is palpable. immunosuppressive treatment and in those patients with immune 42 .

B-cell maturation is arrested at the pre -B-cell stage. They are highly protein bound drugs 5. 9-Macrocephaly :1. Selective IgA deficiency is the commonest form of inherited Ig deficiency (Incidence about 1 in 800). In XLA or Bruton’s agammaglobulinaemia. Is defined as Head circumference > 1 SD ab ove mean 2. 43 . the incidence is higher in patients with chronic lung disease suggesting that IgA deficiency may be associ ated with increased respiratory infections. will cause an increase in renal blood flow 4. which leads to dry cough in many patients. Although usually asymptomatic. The commonest form of immunoglobulin deficiency is however. measles or other viral infections. they will increase renal blood flow and thus GFR.PEDIATRICS EXAMINATION REVIEW complex mediated glomerulonephritis -a major factor is decreased levels of complement. Is associated with neurofibromatosis Teaching Notes for Question 9 Theme: Microcephaly / Macrocephaly macrocephaly:Head circumference (HC) > 2SDs above mean microcephaly: HC > 2SDs below mean Causes of Macrocephaly : Causes of Microcephaly : Familial Familial Achondroplasia Congenital infection Canavan’s disease Fetal alcohol s yndrome Neurofibromatosis Cornelia de Lange syndrome Osteopetrosis Angelman syndrome Tay Sachs disease Edwards and Patau syndr ome Soto’s syndrome Rubenstein Taybi syndrome. Is associated with Cornelia de Lange syndrom e 4. * They are generally poorly protein bound and readily removed by hemodialysis. transient hypogammaglobulinaemia of infancy. in a patient with renal artery stenosis. Indications include congestive cardiac failure in a patient with renal artery stenosis Teaching Notes for Question 8 Theme: ACE inhibitors * ACE inhibitors cause a fall in afterload or SVR. due to an increase in bradykinin levels 3. which leads to hypotension (especially on the first dose) but may increase cardiac output in those with heart failure. * They also prevent the breakdown of bradykinin. Selective IgA deficiency can also occur after phenytoin and barbiturate administration as well as post toxoplamosis. * Although in the normal kidney. Can be inherited 5.ACE inhibitors: 1. This presents between 6 -12 months when the maternal antibody levels wane and the infant’s own humoral system is not yet fully developed. thus they are contraindicated in patients with renal artery stenosis. Some patients with “selective” IgA deficiency may also have deficiencies in IgG2 and IgG4. Is associated with high intelligence 3. May lead to a side effect of dry cough. 8. suggesting that Btk may be required to couple the intracellular signaling events mediated by the pre -B cell receptor. In the normal kidney. they reduce GFR and can precipitate acute renal failure and flash pulmonary edema. They cause hypotension via a drop in systemic vascular resistance 2.

The fluid entering the DCT is iso -osmolar 3. The GFR of a child reaches adult values (corrected for weight) by the age of one year 2. Motor neurone disease. Reidel's thyroiditis. and during this process. The aetiology of motor neurone disease is not clear. Under the influence of ADH. there is near -complete absorption of glucose and amino acids 5. Graves disease. In the proximal tubules. GFR i s corrected for surface area. In myasthenia. the ultrafiltrate is modified: There is preferential absorption of sodium and bicarbonate and near complete absorption of glucose and amino acids in the proximal tu bule. Hashimoto thyroiditis. There are approximately one million nephrons in each kidney Teaching Notes for Question 10 Theme: Normal renal physiology The GFR reaches adult levels by 3 years. Kaposi Sarcoma 4. 12-The following can cause cardiomyopathy in HIV disease:1. Lymphoma 3. CMV infection 5. Glomerular filtration begins at 9 weeks of intrauterine life. 2. there is increased permeability to water in the DCT 4. 4. but renal function is not necessary for normal intrauterine hemostasis. 3. 5.PEDIATRICS EXAMINATION REVIEW 10-Regarding normal renal physiology: 1. not weight. HIV of the heart 44 . Myasthenia gravis. Mycobacterium tuberculosis 2. 11-The following conditions occur due to the presence of circulating antibodies: 1. The proximal tu bule isotonically reabsorbs two thirds of the glomerular filtrate. there are circulating antibodies against acetylcholine receptors at the neuromuscular junction. The glomerulus filters approximately 180 litres of fluid daily. Teaching Notes for Question 11 Theme: Auto antibodies Reidel's thyroiditis is probably viral in origin. Formation of nephrons is completed at birth. but functional maturation does not take place until later.

As pressure in the systemic circulation rises. A pericardial effusion carries a poor prognosis in HIV. X-linked hyper-IgM (type 1) is caused by defects 45 . although TB. Is due to a failure of Ig isotype switching Teaching Notes for Question 15 Theme: X-linked hyper IgM syndrome The hyper-IgM syndrome is a rare .Increases emptying.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 12 Theme: Cardiomyopathy in HIV Cardiomyopathy can be caused by HIV itself. Can present with pneumocystis carinii infection 4. 4 . There is normally immediate closure of the ductus arteriosus 2.X-linked hyper IgM syndrome :1. However. Is associated with normal levels of IgG 3. The ductus arteriosus can take up to 3 months to close in normal neonates.Gastric emptying is slowed by: 1. Domperidone 4. Kaposi sarcoma. HIV cardiomyopathy is linked to CD4 count and HIV encephalopathy. Is due to a defective CD40 ligand 5. The ligamentum teres is the remnant of the umbilical vein 4. this is due to pericardial effusions rather than cardiomyopathy. Causes defective B cell development 2.3. 13. toxoplasma. Supine posture 2. synthesis of bradykinin and prostacyclins is inhibited . Hypoxia-induced vasoconstriction is the mechanism of closure of the ductus arteriosus 3.5 . breathing results in a marked decrease in pulmonary vascular resistance . inherited immune deficiency disorder resulting from defects in CD40 ligand/CD40 interaction. or other infections such as CMV. thus causing closure of the ductus arteriosus . Ranitidine 5.No effect 14. 15. Regression of right ventricular hypertrophy occurs postnatally 5. As the pO2 rises. Gastric distention 3. and lymphoma can cause an apparent cardiomegaly on a chest radiograph.The following is true regarding changes in the fetal cardiovascular system after birth: 1. shunt through the ductus arteriosus reverses. Inferior vena caval pressure falls after birth Teaching Notes for Question 14 Theme: Cardiovascular changes after birth Occlusion of the umbilical cord removes this low resistance cap illary bed from the circulation. Cisapride Teaching Notes for Question 13 Theme: Gastric emptying 2. hence there is increased pulmonary blood flow returning to the left atrium raising the pressure in the left atrium causing the foramen ovale to close .

and T-cell numbers. Patients are susceptible to infection with extracellular bac teria as well as intracellular pathogens . 17. Diagnosis is confirmed by identifying the defective gene . Multiple MRI scans Teaching Notes for Question 17 Theme: Iatrogenic cataracts A cataract is simply any loss of the transparency of the lens of the eye. decrease Na+ and water excretion. although there are high levels of serum IgM which can be produced without T-cell help. There is an increased incidence of autoimmune diseases and malignancies including adenocarcinomas of the liver and biliary tract . liver cells contain angiotensin converting enzyme. Laboratory studies usually reveal normal B. The interaction of CD40L on T-cells and CD40 on B-cells is essential for B-cell activation and proliferation. Systemic steroids 2. kidneys secrete renin. CD40L/CD40 interaction is also necessary for optimal activation of macrophages by T -cells. Prolonged general anaesthesia 3. however neutropenia is common. the loss of interaction between CD40 and its ligand results in impairment of T-cell. Unfortunately.PEDIATRICS EXAMINATION REVIEW in the CD40 ligand expressed on activated T-cells. B-cell and macrophage function. In X-linked hyper-IgM. 4. Total body irradiation 5. Lungs secrete angiotensinogen. These patients are particularly prone to opportunistic lung infection s caused by Pneumocystis carinii. Autosomal recessive hyper -IgM (type 2) is caused by defects in the CD40-activated RNA-editing enzyme. Campylobacter.A cataract may be induced accidentally by the following treatments: 1. isotype switching and formation of germinal centres (which are required for affinity maturation of immunoglobulin). This enzyme is required for immunoglobulin isotype switching and somatic hypermutation in B -cells. 3. IgM levels are elevated but IgG and IgA levels are low. rotavirus . the T-dependent B-cell response is defective . · Renal sympathetic nerves and intrarenal baroreceptor also control renin secretion . 46 .activation-induced cytidine deaminase. theoretically) Chlorpromazine Thalidomide Busulphan Gold. or Cryptosporidium. Treatment is supportive with IVIG and antibiotics . 2. including : Systemic steroid administra tion Irradiation (including multiple CT scans. Renin secretion is controlled by the macula densa. Teaching Notes for Question 16 Theme: Renal system · Angiotensinogen is from liver and the converting enzyme is in the lungs . 5. cataracts may form as a result of many medical treatments. 16. Baroreceptors are the principal sensors for controlling sodium secretion and reabsorption. In reduced sodium states. IgA or IgE production . Changes in aldosterone levels allow control of the re -uptake of 50% of sodium originally present in the glomerular filtrate. Boys present with recurrent sinopulmonary infections and chronic diarrhea often caused by Giardia . both increased activity of renal sympathetic nerves (leading to constricting afferent arterioles) and reduced glomerular capillary pressure. The subsequent macrophage dysfunction contributes to the profound immunodeficiency seen in these patients .Control of absorption: Which of the following statements are true? 1. Massive blood transfusion 4. and there is a lack of IgG. Therefore.

Contains NaHCO3 3. Wilm's tumour and genitourinary malformations. Wilm's tumour and ambiguous genitalia. Surfactant production is suppressed if the baby is hypothermic.PEDIATRICS EXAMINATION REVIEW 18.Aniridia. Is released from the gall bladder in response to the hormone gastrin 4.Also called the cerebro-hepato-renal syndrome. Salts are mostly reabsorbed in the large bowel 47 . Production is increased during a stressful event like hypothermia 5.Bile: 1. Betamathasone given to the mother improves surfactant production in the pre mature baby Teaching Notes for Question 18 Theme: Surfactant Surfactant production begins at 20-22 weeks. 19. Meckel Gruber syndrome 4. Most infants die in the first 6 months. hypoxic.Regarding surfactant:1. Drash syndrome 3. Testosterone stimulates surfactant production 4. Noonan's syndrome 5. Contains salts that are only water soluble 5.Nephropathy. hence hyaline membrane disease is more common in males than females and more common in infants of diabetic mothers. Lethal. Is secreted by the bile canaliculi 2. Often large ballotable kidneys with multiple cysts are present at birth. betamethasone has an identical effect on lung maturation. Meckel -Gruber Syndrome . acidotic or hypoglycemic. Production begins at 30 weeks 2. The hormones testosterone and insulin inhibit surfactant production. Zellwegers syndrome 2. Drash Syndrome .The following syndromes commonly have associated renal manifestations: 1. Chromosome 11p deletion . Although dexamethasone is more commonly used. Chromosome 11p deletion Teaching Notes for Question 19 Theme: Syndromes with renal involvement Zellweger's syndrome .Occipital encephalocoele and renal cysts. It is produced by Type II pneumocytes in the walls of the bronchi 3. 20. It is produced by Type II pneumocytes which are in the walls of the a lveoli.

* VSD (increased RV volume overload). antibiotic treatment are not contraindi cations to vaccines. lung and kidney. when it occurs. 22. Pulmonary hypertension Teaching Notes for Question 22 Theme: Widely split 2nd heart sound Increased normal splitting (wider on inspiration ) of the 2nd heart sound occurs when there is: * any delay in RV emptying as in RBBB (delayed RV depolarization ). and this is done in the terminal ileum. bile pigments. Normal children 2. * pulmonary stenosis (delayed RV ejection). This can be due to: * delayed LV depolarization (LBBB). Severe aortic defect 4. This allows them to stabilise fatty emulsions. Atrial septal defect 5. minor illness . which means that they are both water and fat soluble. 21. bile salts. Fixed splitting (no respiratory variation) is caused by an ASD where equalization of volume loads between the two atria occurs through the defect . * delayed LV emptying (severe AS.though the machinery murmur tends to obscure S2). Live vaccines are not given to children on prolonged steroid therapy 4.Wide splitting of second heart sound may be evident in: 1. chronic diseases of heart. NaHCO3. 48 . * Bile salts are amphipathic. lecithin and chole sterol. fever over 40. persistent screaming. it is most often due to complete RBBB . or serious neurological symptoms. however.. Volume overload due to partial anomalous venous drainage (PAPVD) 3. Stable neurological conditions. and * mitral incompetence (earlier aortic valve closure because of more rapid LV emptying). * Bile consists of. Severe malnutrition is a contraindication for BCG Teaching Notes for Question 21 Theme: Immunisation Live vaccines are contraindicated 3 weeks before and 6 weeks after administration of Immunoglobulins.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 20 Theme: GI * Bile is produced by hepatocytes and secreted into biliary canaliculi. P2 occurs first and splitting occurs in expiration. Live vaccines are contraindicted within 3 weeks of another live vaccine administration 2. In the case of reverse splitting .5 degrees C. It is stored in the gall bladder and gall bladder contraction is stimulated by cholecystokinin (CCK). coarctation of the aorta ) or * increased LV volume load (large PDA.The following are true regarding vaccine administration in children: 1. Splitting of the first sound is not usually detectable clinically. Malnutrition. Severe febrile illness is a contraindication for DPT 5. * 80% of bile salts are reabsorbed.Contraindications to pertussis vaccination include hypersensitivity to the vaccine and a seve re reaction such as shock. MMR can be given a week after administration of IV immunoglobulins 3. Other contraindications to live vaccines include –steroid administration / immunosuppressive therapy / severe egg allergy/ immunodeficient conditions.

2. All have the alpha-beta heterodimeric T cell receptor 4. resulting in apoptosis of unwanted cells. Emergency treatment of hyperkalaemia 1. IV calcium gluconate.PEDIATRICS EXAMINATION REVIEW 23. 5. Peritoneal or hemodialysis. 2.Calcium gluconate stabilizes the myocardium by antagonising the effect of potassium on the heart.Regarding hyperkalaemia :1. IV sodium bicarbonate. Widening of the QRS complex. The two types of TCR are called TCR-1 (gamma-delta) and TCR-2 (alpha-beta). Continuous salbutamol nebulisers or IV salbutamol. 4. Note. Tall P waves are seen on ECG 2. 4. Ion exchange resin. 95% die in the thymus 3. If acidosis is corrected. Ventricular fibrillations. or gamma and delta) linked by disulphide bonds and is found at the cell surface associated with a complex of other polypeptides known collectively as CD 3. The TCR is a heterodimer of two chains (alpha and beta. 6. Are derived from a precursor cell in the bone marrow 2. 24. Peaked T waves. ventricular tachycardia. Glucose-insulin infusion. potassium shifts back into the intracellular f luid Teaching Notes for Question 23 Theme: Hyperkalaemia ECG changes of hyperkalaemia are 1. Continuous salbutamol nebulisers should be started immediately on diagnosis 3. TCR-2 cells recognise peptide fragments associated with MHC molecules . 3. 6. Maturation occurs in the thymus . 5.T lymphocytes:1. 49 . Calcium gluconate exchanges for potassium and enables its excretion 5. Are prominent in type II hypersensitivity reactions Teaching Notes for Question 24 Theme: T lymphocytes T lymphocytes comprise 70-80% of the lymphocyte population and are haematopoietic cells. TCR-1 cells are thought to have a restricted repertoire and to be mainly non -MHC restricted. ST depression. Prolonged PR interval. and involves a complicated series of positive and negative selection steps coordinated by the thymocytes and dendritic cells within the thymus. They are produced in bone marrow and paracortical regions of lymph nodes and spleen. Are usually activated by intact protein antigens 5. 3. IV calcium resonium should be commenced 4. It does not lower serum K+ levels.

Other causes of retinitis pigmentosa are:Freidrich’s Ataxia. Medication also occasionally used. May lead to avoidance 4. or dramatic reenactment. Lack of energy. plasma phytanic acid levels fall with an improvement in the neurological signs. sometimes using play. Is affected adversely by radical weight change Teaching Notes for Question 26 Theme: Refsum’s Disease Refsum's disease is an autosomal recessive disorder caused by defective peroxisomal oxidation of phytanic acid.Post-traumatic stress disorder :1. together with feelings of detachment or emotional numbness or blunting. panic attacks. cerebellar ataxia. storytelling. Is associated with feelings of detachment Teaching Notes for Question 25 Theme: Post-traumatic stress disorder Post traumatic stress disorder is characterised by a delayed or protracted response t o a stressful situation or event. anosmia. It is totally unrelated to Hurler’s sy ndrome. Is one of the causes of retinitis pigmentosa 5. By limiting intake of meat (animal fat) and green vegetables. Alports syndrome and Laurence-Moon-Biedl sydrome. and sensory and hypertrophic interstitial neuropathy with preservation of intellect. Is an immediate response to a traumatic event 2. Kearns –Sayre syndrome. Toxic accumulation of phytanic acid causes adolescent onset progressive retinitis pigmentosa.PEDIATRICS EXAMINATION REVIEW 25. Often involves ‘flashbacks’ 3. Plasmapheresis has been used if the patient's clinical condition rapidly worsens. cataracts. anxiety. depression. 26. Family therapy sometimes useful. Can sometimes be treated by play therapy 5. The mutation is in the phytanoyl -CoA hydroxylase (PAHX) gene on chromosome 10p13. 50 . Symptoms include reliving of the trauma or event in ‘flashbacks’ or dreams. Is inherited as an autosomal dominant disorder 2. deafness. puppetry.Refsum’s Disease: 1. Is associated with icthyosis 3. Ushers syndrome. This occurs because the mutations are not absolute in eliminating function and because omega-oxidation can also reduce phytanic acid levels. Treatment: intensive individual therapy. The disorder is asso ciated with icthyosis (a desquamative skin disorder) and cardiac arrhythmias. Cerebellar dysfunction also manifests more prominently with rapid changes in the patient's weight (particularly rapid weight loss) and hence it is reco mmended that a stable body weight is mantained. Avoidance of people or situations associated with trauma. abetalipoproteinaemia . depending on symptoms encountered. Is caused by the same enzyme as Hurler’s disease 4.

UTI’s. diverticulitis and abdominal herniae . 5. although sustained VT is uncommon .Disseminated intravascular coagulation: 1. Clinical presentations include hypertension . Strenuous activity is proscribed in all patients Teaching Notes for Question 28 Theme: Hypertrophic obstructive cardiomyopathy In hypertrophic cardiomyopathy . haematuria. Liver cysts are seen in approximately 10%. It may be associated with aortic stenosis. Treatment with beta-blockers and amiodarone may be of benefit as may implantation of a defibrillator . Is usually associated with a high fibrinogen level 2.000. Runs of non-sustained VT are found in one-fourth of patients of HCM patients . It can present in the newborn. it is a harbinger of sudden cardiac death . affecting between 1:1. its overall predictive value in identifying patients at high ri sk of sudden death is quite limited . It can present at any time from early infancy. 2.000 of the population. however.PEDIATRICS EXAMINATION REVIEW 27. Runs of Non sustained ventricular tachycardia on Holter have a high predictive value for sudden cardiac death 4. Is usually associated with a normal thrombin time 51 . 28-The following statements are true regarding Hypertrophic obstructive cardiomyopathy:1. abdo mass or pain and polycythaemia. and PKD2 has been located to chromosome 4. In some. Presentation in childhood signifie s worse prognosis. the degree of outflow obstruction and the risk of sudden death depend on the gene defect . as well as renal impairment. The genetic mutation of type I has been linked to chromosome 16. Progresses to dilated cardiomyopathy in 10% cas es 5. There is no correlation betreen these two features however. Prognosis is worse in children 3. Other associations include mitral valve prolapse . 29. 4. The prognosis is related to the degree of left ventricular outflow gradient 2. The genetic mutation of type 1 is on the short arm of chromosome 16 (PKD1 gene). about 10% have pancreatic cysts and about 5% have Berry aneurysms . Over 50% have liver cysts . The incidence is about 1:10.The following are true of adult polycystic kidney disease: 1. 3. aortic incompetence .000 and 1:2. Teaching Notes for Question 27 Theme: Adult polycystic kidney disease APKD is an autosomal dominant condition .

the storage form of carbohydrate. Removal of glucose (glycogenolysis) 52 . renal failure. each residue being added from an activated donor molecule. HIV and varicella zoster. Is characterised by arterial hypertension. is found predominantly in liver and muscle as cytoplasmic granules. May be associated with cytomegalovirus or varicella zoster infections 4. Cardiac tamponade can occur after both penetrating and blunt injuries to the chest. raised JVP. Occurs only after penetrating injuries to the heart. at every 10 residues or so.PEDIATRICS EXAMINATION REVIEW 3. Branches in the glycogen molecule are produced by 1. and is characterised by hypotension . muffled heart sounds and decreased voltages in all ECG leads . * Liver disease can also cause DIC due to release of t hromboplastins from damaged liver cells and reduced concentrations of antithrombin and protein C. May be associated with formation of microthrom bi. Deficiency of muscle phosphorylase. May occur in liver disease 5. Chains are formed by Alpha 1. Teaching Notes for Question 30 Theme: Cardiac tamponade. McArdle’s Disease results in fasting hypoglycaemia and hepatomegaly Teaching Notes for Question 31 Theme: Glysogen metabolism Glycogen. Adrenaline is an important stimulus for glycogenolysis in the liver during exercise 4. * Investigations show a prolonged thrombin time. in particular gram-negative and meningococcal septicemia. * DIC may be associated with microthrombin causing skin lesions. Should be treated with aspiration using a needle inserted via the second intercostal space. 30-Cardiac tamponade: 1. may result in an impaired exercise tolerance 5. 3. 2. 31-Regarding glycogen and its metabo lism: 1. UDP-glucose. are formed by 1. a low fibrinogen and ra ised D-dimers or FDPs. Branches. prothrombin time and activated partial thromboplastin time (aptt). Kussmaul’s sign. Glycogenolysis in the liver releases glucose-6-phosphate into the blood stream 2.6 glycosidic bonds formed by a separate enzyme.4 -glycosidic linkages 3. Glycogen is a large branched polymer of glu cose residues.4-glycosidic bonds. gangrene or cerebral ischemia but heparin therapy is usually not indicated as it can aggravate the bleeding tendency. The central venous pressure is raised. thrombocytopenia. mid-clavicular line. 5. Is characterised by increase ECG voltage across all leads. by severe falciparum malaria infections and by viral infections including CMV. 4. so should be treated with intravenous heparin Teaching Notes for Question 29 Theme: Disseminated intravascular coagulation * Disseminated intravascular coagulation (DIC) m ay be caused by bacterial infections.

e. Sickle cell disease Teaching Notes for Question 33 Theme: Somatic gene therapy Somatic gene therapy (i. Neonatal infection is more likely if the mother has concomitant HIV infection as well as HCV 4. as this form of gene therapy is unlikely to restore the levels of the protein to normal. 32-Concerning Hepatitis C Virus: 1. starvation) and adrenaline (muscle. Thalassaemia 3.6glycosidic bonds by a separate debranching enzyme. It is spread by blood contact especially via blood transfusion and IV drug abuse. Adenosine deaminase deficiency. Patients with diabetes mellitus require controlled release of insulin and gene therapy is unlikely to provide the answer. Adenosine deaminase deficiency 2.7% of the UK population is affected. sometimes in combination with ribavirin but response depends on viral genotype. 33-Which of the following conditions would be suitable for somatic g ene therapy:- 1. Co infectin with HIV results in more rapid and severe progression to chronic liver disease. 1% of the world’s population is infected 2. exercise). addition of a gene to the somatic cells or tissues rather than the germ line) is ideally suited for monogenic disorders caused by loss of gene function where even partial restoration of the protein will greatly ameliorate the symptoms. Unlike hepatitis B infection is not concentrated in developing countries and 0. Liver transplantation is usually required due to the inadequacy of medical therapies Teaching Notes for Question 32 Theme: Hepatitis C Hepatitis C infects an estimated 500 million people. haemoglobinopathies in particular thalassaemia maj or. The proportion developing chronic infection and hepatocellular carcinoma are similar to HBV infection 3. Sexual and vertical transmission occurs but is much less common. In muscle. type II (McArdles disease) caused by a failure of muscle phosphorylase leading to normal blood glucose but impaired exercise tolerance. Conditions that stimulate glycogenolysis inhibit glycogen synthesis. Glucose-1-phosphate is then converted to glucose -6phosphate. and vice versa. there is no phosphatase trapping glucose -6-phosphate within the cell.4-glycosidic bonds by phosphorylase to produce glucose . Hepatitis C can be treated with interferon alpha. and haemophilia fit these criteria. age.PEDIATRICS EXAMINATION REVIEW occurs by cleavage of alpha -1. The rate of glycogenolysis is hormonally regulated by glycogenolysis (liver. Breast feeding should be discouraged in the HCV +ve mother 5. Haemoplilia A 5. Diabetes mellitus 4. Glycogen storage diseases: type I (von Gierke's disease) results from glucose -6phosphatase deficiency leading to failure of release of glucose from the liver.1phosphate and by cleavage of a -1. In the liver. Massive hepatomegaly and fasting hypoglycaemia develop. a glucose-6phosphatase allows passage of free glucose into the bloodstream. Islet cell transplantation into the portal vein is more promising as it preserves the microenv ironment of the beta cells of the islet and allows controlled temporal release of insulin. 34-In paroxysmal nocturnal haemoglobinuria:- 53 . alcohol intake and degree of liver fibrosis and inflammation seen on biopsy.

The diagnostic test is Ham’s test (acidified serum lysis). Crises of haemolysis can be triggered by infection. 5% Teaching Notes for Question 34 Theme: Paroxysmal nocturnal haemoglobinuria PNH is an acquired clonal defect of cell membranes that makes the cells more sensitive to lysis by comple ment. Cause relaxation of the diaphragm 5. 54 . The disease may transform into acute leukaemia (in approx 5%). incre ase the sensitivity to CO2 and increase diaphragmatic contractility. Infertility is a frequent complication of the orchitis associated with mumps 5. incubation period is 14 -21 days.PEDIATRICS EXAMINATION REVIEW 1.3. They are ino -dilators. The diagnostic test is Ham’s test 5. Venous thrombosis is a recognised complication 4. in unusual sites such as saggita l sinus or hepatic veins). They are bronchodilators. Children are not infectious after the development of parotitis 4. Inhibition of tubular Na reabsorption leads to a naturesis and may precipitate hypokalemia. It is caused by a DNA virus Teaching Notes for Question 36 Theme: Mumps Mumps is caused by an RNA virus. and chronic haemolysis may result in nephropathy. It is often asymptomatic 3. Cause peripheral dilatation 4. The defect is a congenital abnormality of RBC production by the bone marro w 2. Infertility is not common after orchitis. 36-Regarding mumps infection: 1. This results in intravascular haemolysis and haemoglobinuria. 2. The other important complications are CNS infection (aseptic meningitis) and pancreatitis. Hamoglobinuria is a characteristic finding 3. Decreases the pulmonary capillary wedge pressure Teaching Notes for Question 35 Theme: Inotropes and vasopressors 1. 5. Both the LVEDP and PCWP decreases. They cause a weak diuretic effect. There is an increased risk of venous thromboses (esp. Children are infectious for a few days before until a week after the parotiti s. Cause bronchodilation 3. Transforms into acute leukaemia in approx. 4. 35-Phosphodiesterase inhibitors: 1. In 30-40% of cases the infection is asymptomatic. The incubation period is 7-14 days 2. Have an anti-diuretic effect 2.

TGF-beta 5. rubella and polio are contraindicated in HIV +ve children 4. 38-Regarding immunisation in the UK: 1. They also promote the production of opsonising antibodies. Promotion of immunoglobulin isotype class switching 3. irrespective of prematurely.PEDIATRICS EXAMINATION REVIEW 37-The following are known functions of T helper cells : 1. Family history of febrile convulsion is not a contraindication to immunisation. Induction of the maturation of immature dendritic cells Teaching Notes for Question 37 Theme: Functions of T helper cells Perforin is produced by cytotoxic T lymphocytes (CTL) to kill target cells. IFN-g. Premature babies should be immunised according to the recommended schedule from 2 months after birth. They may not be able to produce a normal immune response to live vaccines and could potentially suffer from severe manifestation such as disseminated infection with BCG or paralytic polio from vaccine virus. Teaching Notes for Question 38 Theme: Immunisations in childhood Prematurity. More recently. However it should be noted that Polio is secreted for longer periods in HIV positive patients as compared to other people. chronic lung disease. Prolonged inconsolable crying to previous immunisation is a definite contraindication. and 4 months afterbirth. HTL can be sub-classified into TH1 and TH2 cells. Patients on active chemo or radiotherapy or within 6 months of termination of such treatment are contraindicated for live vaccines. Th2 cells secrete IL -4. congenital heart disease. TNF-beta and are important for cell mediated immunity. Production of suppressive cytokines such as IL -10. Live vaccines should be avoided within 6 months o f termination of lymphoma treatment 5. Measles. Th1 cells secrete pro -inflammatory cytokines such as IL-2. In general. No harmful effects have been reported with MMR and polio in HIV +ve individuals. The most common indication is failed Kasai procedu re 55 . Prolonged inconsolable crying or high pitched screaming more than 4 hours is classified as a severe reaction to immunisation and is a contraindication to sub sequent immunisation with same vaccine. Studies have shown that antibody response and adverse events are not significantly different in preterm and term infants immunised 2. Helper T lymphocyt es (HTL) are usually CD4+ T-cells. Family history of febrile convulsion following immunisation is a relative contraindication 3. Production of perforin 4. The first dose of vaccines is given at a correct ed age of 2 months in case of premature delivery 2. further sub -populations of CD4+ T cells have been identified. Th3 and Tr1 are not yet well defined but the former secrete predominantly IL-10 while the latter secrete mainly TGF-beta It must be remembered that this differential cytokine expression is not absolute. asthma. 39-Regarding paediatric liver transplantation: 1. termed Th3 and Tr1 cells. IL-5 and IL-10 and are important for humoral responses. Production of T cell growth factors such as IL-2 2. mumps. and Downs syndrome are associated with an increased risk of complications from infectious diseases and they should be immunised as a matter of priority. TNF-alpha .3.

Early complications include fluid shifts. and hypertension. 3. 4.PEDIATRICS EXAMINATION REVIEW 2. It may be caused by injury to the superior cervical sympathetic ganglion. There is an association with coarse facies. The condition is X-linked 5. Vascular complications. Orthotopic (same species) transplants are surprisingly successful especially with improved immunosuppression and better preservation of the organ (up to 18 hr ex vivo with <2% primary nonfunction). Organ rejection tends to occur after the early phase along with infection. also may be an ominous early problem. electrolyte imbalance . There is a low incidence of atopy due to defective IgE function 4. Teaching Notes for Question 40 Theme: Horner’s syndrome. There is a markedly raised IgE level 2. Signs of chronic liver disease persist despite improvement in liver function 5. Ptosis occurs because of loss of innervation to the smooth muscle portion of the levator palpebrae superioris. FK506 has superceded tacrolimus as an immunosuppressant 4. so does the child’s growth stigmas of chronic liver disease resolve. tacrolimus is associated with lower rates of acute and chronic rejection and reduced use of corticosteroids. The next most common indications are metabolic liver disease and acu te hepatic necrosis. Organ rejection is an early post operative complication Teaching Notes for Question 39 Theme: Liver transplantation Paediatic liver transplantation is an uncommon procedure (about 500/year in US) but the most common reason is following a failed portoenterostomy (Kasai) procedure following extrahepatic biliary atresia. Pupillary dilatation occurs because of loss of innervation to the iris sphincter muscle. Tacrolimus and FK506 are the same drug. kyphoscoliosis. renal dysfunction. Compared with cyclosporin. It is associated with ipsilateral ptosis (paralysis of levator palpebrae superioris). such as thrombosis of graft vessels. 40-The following statements are correct regarding Horner’s syndrome: 1. 2. and eczema 3. meiosis (paralysis of dilator pupillae muscle) and facial and upper limb anhidrosis. 5-yr survival rates for children after orthotopic liver transplantation are around 30% 3. Most recent figures are 81% at 1 year and 77% at 5 years. May be associated with ophthalmoplegia in the territory of the IIIrd cranial nerve. Steroids and either cyclosporine or tacrolimus are standard therapy to prevent rejection. May be associated with loss of sweating on the ipsilateral arm. Horner's syndrome may be caused by any injury to the sympathetic chain in the neck. “Cold abscesses” occur 56 . 5. osteoporosis. The somatic component of the IIIrd nerve is unaffected and thus ophthalmoplegia is not a feature 41-In HIE or Job’s syndrome : 1. As liver function improves.

* Beta-adrenergic drugs. Is associated with splenomegaly at diagnosis in about 50% of patients Teaching Notes for Question 43 Theme: Chronic lymph atic leukaemia Chronic lymphatic leukaemia (CLL) is due to an accumulation of mature lymphocytes in the peripheral blood. but one third of patients die of causes other than their CLL. Benzylpenicillin 57 . Has a median survival of less than 5 years 5. Prostacyclin Teaching Notes for Question 42 Theme: Pulmonary circulation The PVR is increased by * Hypoxia. bone marrow. Patients develop recurrent sinopulmonary and cutaneous infections that tend to be much less inflamed than appropriate for the degree of infection (cold abscesses). The IgE level is mark edly raised in these patients 42-The following increases the pulmonary vascular resistance 1. and splenomegaly is seen in 50% at diagnosis. * Alpha-adrenergic drugs. Is asymptomatic at diagnosis in less than 10% of patients 3. The PVR is decreased by * Vasodilators. Is associated with smear or smudge cells in the peripheral blood 2. hypercarbia. * 5HT. Anemia 4. It is asymptomatic at diagnosis in over 30% of patients. acidosis. It is often a slowly progressive disease and should not be treated in the early stages. vasoconstrictors. Median survival is 3-5 years. The lowest PVR is at the FRC 43-Chronic lymphocytic leukaemia : 1. histamine. * Parasym nervous sys * Prostacyclin Effect of respiration: PVR varies with lung volume in a U -shaped curve. liver and lymph nodes. Maximal expiration 3. spleen. Maximal inspiration 2. 44-The following agents have good activity against Bacteroides fragilis 1. Ach. Smear or smudge cells are often seen in the peripheral blood which represent the fragile m alignant cells which have been squashed as the blood film is spread. osteoporosis. Salbutamol 5. Should be treated aggressively in the early stages of the disease 4.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 41 Theme: HIE or Job’s syndrome HIE (HyperIgE syndrome) or Job’s syndrome is an autosomal dominant condition characterised by coarse facies. atopy and eczema. kyphoscoliosis.

Vitamin K is also fat soluble and is also taken up by fat absorbing processes in the gut. Metronidazole 5. Amphipathic phospholipids and bile salts convert micelles into lipid droplets ready for absorption. Leukotriene inhibitors are associated with the development of Churg -Strauss . 47-Regarding overdose. Chloramphenicol Teaching Notes for Question 44 Theme: Anaerobic Infections B fragilis produces a â-lactamase which is sensitive to inhibitors. 5. pathophysiology In asthma. Trypsin. D and E are taken up by fat absorbing processes in the gut. chymotrypsin. Digoxin 2. Piperacillin / tazobactam 3. It also produces a cephalosporinase. Propranolol 58 . 2. 4. cellulose and glycogen are broken down for absorption by salivary and pancreatic amylases. 5. IL-5 is produced by T helper cells (TH-2) and recruits eosinophils from the marow. Blocking IL -5 will therefore be therapeutic. The eosinophils to the lung are recruited from the bone marrow 2. Leukotriene inhibitors are associated with Churg -Strauss syndrome and causality can not be excluded. haemodialysis would be effective in removing drug in: 1. 3. carboxypeptidases and amino peptidases are responsible for the breakdown of proteins for absorption. Bronchial hyper-responsiveness (BHR) does not have to be present. Amitriptyline 4. The three known fat soluble vitamins A. Teaching Notes for Question 45 Theme: Gastrointestinal system Amylases attack starch.PEDIATRICS EXAMINATION REVIEW 2. About 9 litres of water enters the gut each day. 46-In asthma:1. 45-Digestion and absorption: Which of the following statements are true? 1. Pethidine 3. cellulose is indigestible dietary fibre. but it does not hydrolyse cefoxitin (cephalosporin with anaerobic activity). by definition bronchial hyper -responsiveness must be present. Micelles are smaller structures made from lipid droplets by these agents. Cefoxitin 4. Blocking interleukin-5 maybe therapeutic 4. Steroids are contra-indicated if the patient is a diabetic 3. Teaching Notes for Question 46 Theme: Asthma. The glucose-containing polysaccharides starch.

meprobamate. Chromatin plays no role. histone H1. ethylene glycol and lithium. 5. Condensation of chromatin varies at different stages of the cell cycle and with transcriptional activity. Teaching Notes for Question 49 Theme: Gene regulation The association of double-stranded DNA with histones (and other non-histone) proteins forms chromatin. DNase I hypersensitivity sites are usually indicative of regions of active chromatin. chloral hydrate. Haemoperfusion is effective for short and medium acting barbiturates. tricyclics and lipid soluble beta-blockers have a very large VD. Local humidity and air movement do not significantly influence heat loss. phenobarbitone. Digoxin. A fifth protein. H2B. Activated charcoa l should be given for aspirin. Sweating can increase body evaporative water loss from 0. 49-In gene regulation:1. 4. 2.5 litres per day and eliminate 2. Nucleosomes consist of eight proteins . Elaborate folding and protein –histone interactions organise the chromatin fibre (made up of the nucleosomal DNA) into a h igher order structure with varying degrees of compaction. The stability of mRNA is entirely dependent on its length. H3 and H4 around which the DNA coils. dapsone. pacing/glucagon/isoprenaline for propranolol. 3. betablockers for arrhythmias from tricyclics. Drugs for which haemodialysis is effective include salicylates. Changes in muscle activity are the main source of heat production for temperatu re control. Salicylate Teaching Notes for Question 47 Theme: Haemodialysis . quinine. Humidity controls evaporation and therefore the effectiveness of sweating. DNAse hypersensitive sites are often associated with gene control regions such as promoters and enhancers as the activating transcrip tion factors bound to 59 . The skin is a good thermal insulator whose effectiveness is controlled through blood flow. Regular nucleosomal spacing can be interrupted by transcription factors and other DNA bindin g proteins resulting in the creation of a nuclease hypersensitive site. DNase I hypersensitivity is a method used to analyse the state of chromatin at a particular site on a chromosome.two copies each of H2A. phenobarbitone. and theophylline. 5. carbamazepine. and theophylline.drugs The volume of distribution (VD) of the drug determines how effective dialysis will be in removing it from the body. The blood flow through skin is controlled through vasodilator sympathetic nerves whose firing rate is increased in response to heat. which increase their firing rate in re sponse to cold and decrease with heat. These nucleosome struc tures together with DNA form a repeat unit of approximately 200 nucleotide pairs and are very regularly arranged along the chromosome. 4. 2. helps pack the nucleosomes together to increase compaction. All convective losses increase with air movement.6 to over 4. naloxone for pethidine. 3.4 Mcal heat from the body. methanol. opiates. DNA regions that are assembled into regularly spaced nucleosomes are m ore resistant to cleavage by this enzyme than are regions that lack nucleosomes due to the binding of proteins to the DNA.PEDIATRICS EXAMINATION REVIEW 5. More effective supportive measures are antidigoxin antibody/atropine/correction of hypokalaemia for digo xin. 48-Heat inputs and outputs: Which of the following statements are true? 1. Transcription factors control expression by binding to specific DNA sequences. Teaching Notes for Question 48 Theme: Thermoregulation Skin blood flow is largely controlled by sympathetic vasoconstrictor nerves. Transcription levels determine protein levels .

Which one of the following investigations is the most relevant for her future management? 1. Transcription factors are proteins that bind to specific DNA target sequences and then act to enhance transcription. These areas are refractory to gene expression.P was concerned about this and has addressed this issue with her and her parents. efficiency of translation will be another factor determining the level of protein produced. Secondly. are physically linked to the gene. making it accessible to digestion by DN ase 1. Plasma leptin 5.PEDIATRICS EXAMINATION REVIEW these regions displace nucleosomes or alter their structure. They alter chromatin structure by displacing the nucleosome histones and 'opening up' the DNA. Fasting serum cholesterol/triglycer ide 2. while cis refers to the sequences that are immediately upstream of the gene i. They may not bind directly to the polymerase. She is the only child of her parents. the heterochromatic regions. Serum adrenocorticotrophic hormone (ACTH)/cortiso l 4. Her weight is 49 kg and her G. Trans means 'from elsewhere'. 50-Sarah is eight years old and she was referred by her G. This is achieved by various protein complexes which use energy to displace histones or which modify histone tails . The role played by length of mRNA is unknown but it is proba bly not a major factor determining stability of the transcribed sequence. The complex mechanisms of ARE -mediated post-transcriptional regulation are still being revealed. Specific sequences in the message seem to be involved in stabilisation. the half life of mRNA. but to this secondary release. However it is important to remember that this is not always the case. for instance. because the protein is usually encoded on a different chromosome from the ones on which they operate. It is not itchy and she is not disturbed by it. together with the presence of alternatively spliced variants can affect the steady state levels of mRNA considerably. pigmented rash on her neck and axilla. Euc hromatin refers to chromatin that is decondensed in interphase and is rich in actively transcribed genes.g.e. The consensus sequence in the ARE is the AUUUA repeat e lement. Some regions of DNA. Some proteins that bind to DNA act as silencers. and in some cases transcription factors may still bind DNA when the gene is not expressed. In this case. The higher order structure of chromatin plays a major role in gene regulation. Finally. 51-Congenital CMV infection: - 60 . They are known as trans-acting factors. Serum thyroid-stimulating hormone (TSH) Teaching Notes for Question 50 Theme: Diabete mellitus Acanthosis nigrans is associated with insulin resistance or poor control and is indicated by a serum insulin/glucose ratio. acetylation of histone tails opens the DNA stru cture and aids in transcription factor binding. to the cell surface after an additional signal. but can interact via associated factors. The ARE triggers destabilization of mRNA when bound by certain proteins. Fasting serum insulin/glucose ratio 3.MK2 (a protein kinase induced by LPS) may inhibit the binding or activity of destabilising proteins associated with the AREs of proinflammatory cytokine mRNAs. Thus DNase hypersensitive sites are often associated with active chromatin. This sequence is found in the 3' noncoding region of many eukaryotic mRNAs particularly those of transiently expressed proteins such as cytokines and growth factors. usually by stabilising the comp lex containing the RNA polymerase.P with a velvety.e. once the mRNA has translocated to the cytoplasm and is translated. expression on the cell surface would not be related to transcription at all. proteins can be stored in the cytoplasm for release. However. The best example is that of the AU rich element (ARE). and are usually made up of DNA containing multiple repeat sequences. Chromatin must be open for transcription to take place to enabl e the various transcription factors and the components of RNA polymerase to gain access. AREs have also been shown to play a role in the stabilization of mRNA . A major factor in dictating the level of messenger RNA (mRNA) in the cell is obviously transcription. consist of very compacted nucleosomes.

Anti-centromere antibodies are seen in up to 90% of patients with the latter. As with several chronic inflammatory diseases. Infection in early gestation carrie s a far greater risk of severe fetal disease. Is associated with an increased risk of ischaemic heart disease Teaching Notes for Question 52 Theme: SLE Kikuchi’s syndrome (histiocytic necrotising lymphadenitis) occurs in young females and presents with pyrexia and cervical lymphadenopathy and is associated with SLE. Only 10% of affected pregnancies have resulting long term sequele at birth 2. Intracranial calcifications are seen in a periventricular distribution Teaching Notes for Question 51 Theme: Congenital CMV. In CMV intra-cranial calcifications are in a periventricular distribution. fingertips and knees (but can occur anywhere). Intravenous immunoglobulins are commonly used in severe disease 4. 53-In scleroderma (systemic sclerosis):1. Hearing loss can deveolp gradually over the first 5 years 4. Little race effect. Diagnosis is by viral isolation from the urine 3. The affected newborn should be treated with ganciclovir 5. Anti-Scl 70 antibodies occur in 50-60% of cases 5. Penicillamine may delay the development of i nternal organ complications Teaching Notes for Question 53 Theme: Scleroderma Most patients' skin softens after 3 -10 years of disease. The most commonly affected sites are the PIPs. Internal organ involvement is usually progressive. The female to male ratio is 3:1 (age 35 -65). There is limited evidence that penicillamine delays the progression of cutaneous 61 . there is an increased risk of IHD. The ANA is positive in about 95% of patients with either form. Ganciclovir is only used if there is CNS involvement. Females are affected three times more commonly than men 3. Calcinosis only occurs in CREST form of the disease 4. Exacerbations may be associated with lymphadenopathy 2. Arthralgia and tiredness are almost universal. Cyclophosphamide and high dose steroid is the first line in life-threatening flares. elbows. Rare in children. lymphadenopathy per se is commonly observed in flares. Severe clinical disease is associated with primary maternal infection in pregnancy. Congenital CMV occurs in approximately 1% of all live births and only 10% of these infections result in clinical symptoms. chorioretinitis or pneumonitis. Raynaud’s phenomenon is the commonest presenting symptom 5. 52-In systemic lupus erythematosis (SLE) 1. Calcinosis (deposits of BCP) is seen in about 50% of patients with limited SS (CREST) and in 5% of those with diffuse SS. Skin tightness may improve with time 2. although progressive digital contractures may occur. There is an increased liability to infections 3.PEDIATRICS EXAMINATION REVIEW 1. Anti-topoisomerase (Scl-70) antibodies are only seen in 20-30% of patients with diffuse disease and in 10-15% of patients with CREST.

Middle East) S. Japonicum (China. Prior to the introduction of USS e xamination. Intercalatum (West/Central Africa) S. Haematobium Urinary schistosomiasis (Africa.then nephrectomy is warranted. If hypertension does develop -which is rare . Schistosoma haematobium infection may lead to an obstructive uropathy Teaching Notes for Question 55 Theme: Schistosomiasis Schistosomiasis is a trematode (fluke) infection. Miracidia hatch and infect freshwater snails (intermediate hosts). which has an incidence of 1:20. Schistosoma haem atobium infection may lead to rectal bleeding 5. Japan . 54-Regarding multicystic dysplastic kidney disease: 1. is incompatible with life. Fever. and nephrectomy was routinely performed. Cercariae penetrate the skin and may lead to an itchy papular rash 3. 4 -8 weeks later cercariae emerge which penetrate skin of Worms reside in vesical plexus Eggs shed in urine Intestinal/liver schistosomiasis Worms reside in mesenteric veins Eggs shed in stool 62 . There is usually 0% differential function on DMSA/ MAG3 imaging 5. Un like most connective tissue disorders. UnilateraI lesions have an incidence of 1:2 500-1:4000 live births 4. 55-Regarding schistosomiasis: 1. Americas) S. However. Intermediate host . It is treated by routine nephrectomy 3. Bilateral disease.snails Definitive host . corticosteroids should probably be avoided in scleroderma as they may precipitate a renal crisis.humans S. CDK was thought to be an uncommon disorder that presented as an abdominal mass during childhood.000. It is a rare anomaly that presents as an abdominal mass in th e neonate 2. Hypertension is a common complication of MCDK that warrants nephrectomy Teaching Notes for Question 54 Theme: Multicystic Dysplastic kidney disease Multicystic dysplastic kidney (MCDK) is a congenital disorder in which cysts of multiple sizes replace the normal renal cortex. SW Asia. Mekongi (Mekong Delta) LIFE CYCLE: Eggs are shed in urine or stool into fresh water. Mansoni (Africa. MCDK is usually a unilateral disorder. it is now apparent that the condition is commoner than was previously supposed and unilateral dis ease often follows a clinically undetectable course. Freshwater snails are the definitive host 2.more severe) S. urticaria and eosinophilia may be seen in the initial illness due to antigen -antibody complex formation 4. Evidence suggests that prophylactic nephrectomy in this condition is not indicated as complications are rare.PEDIATRICS EXAMINATION REVIEW disease and interstitial lung disease.

corpulmonale) or CNS (space occupying lesion. They have a life span of approximately 10 days 2. Immunodiagnostic tests . Platelet concentrates should be stored at around 20 degrees C and the pH kept between 6. Platelet production is controlled by specific cyto kines 3. eggs have terminal spines remember terminal haematuria too) or STOOL (need to concentrate stools by formol -ether method. cord compression) 4. 57-Concerning Salmonella infection: 1. no serious toxi cityct . Infection invariably occurs following oral ingestion of contaminated food products 3. Results in a non-invasive enteric infection 2. hepatosplenomegaly. an increment of 10 000 / ml. Invasion of cercariae: * Swimmer's itch (papular dermatitis) 2.8 .S. x3 doses Praziquantel .PEDIATRICS EXAMINATION REVIEW humans and migrate via the lungs to t he liver as a schistosomule.all species. Statutory notification is required 4. do not tell past vs present infection. single dose (sometimes repeated). They have a lifespan of 10 -12 days thereafter being destroyed in the spleen. Eggs cause most of the pathology: a) Eggs leaving gut/urinary tract cause bleeding b) Eggs remaining cause granulomata and calcify: * Urinary tract . Adult worms develop in 1-3 months in the portal veins and migrate to their final habitats (see above) where eggs are laid. Immune complex disease: * Glomerulonephritis * Nephrotic syndrome DIAGNOSIS: 1. eosinophilia. One pool of platelets gives on average. cough/wheeze. Platelets should be stored between 2 and 6 degrees C 4. calcified bladder.2 and 7. diarrhoea * Can treat with steroids 3.obstructive uropathy.these conditions reduce the risk of a change in morphology of the platelets.colonic polyposis leading to iron deficiency anaemia c) Eggs in the portal tract cause periportal fibrosis (Symmer s fibrosis) leading to portal hypertension (but normal liver function) d) Eggs can embolise to lungs (pulmonary hypertension. Antibiotic treatment is required for Salmonellosis 63 . Platelets can be stored for a maximum of 48 hours 5. a specific cytokine. pseudopapillomas * GI tract . haematobium only. not species -specific. hi-tech. which are regulated by thrombopoietin.colonic polyposis leading to iro 56-Regarding platelets: 1. urticaria. * squamous cell carcinoma bladder. They should also be continuously agitated to encourage gas exch ange. A pool of platelets transfused can produce an increment of 50 000 / ml if platelet consumption is not an issue Teaching Notes for Question 56 Theme: Platelets Platelets are produced from megakaryocytes. PATHOLOGY is caused by: 1.problematic: no indication of severity of infection. TREATMENT: Metriphonate . eggs have lateral spines) 2. Direct microscopy of eggs in URINE (max at 12 noon. Katayama fever: * Acute infection * Fever.

. Is surgically treated by excision of the pyloru s muscle Teaching Notes for Question 59 Theme: Infantile hypertrophic pyloric stenosis Pyloric stenosis occurs more commonly in males with a ratio of 4:1. Is more common in the Afro-Caribbean population 3. Penicillamine 64 . Bicarbonate then diffuses into plasma in exchange for Cl ions. potassium and bicarbonate 5. Small rise of 0. hypochlorhydria. It becomes more frequent and projectile in nature. Chloramphenicol 2. Ultrasound (US) examination confirms the diagnosis. which then dissociates to hydrogen ions and bicarbonate. bilious vomiting 4. Is transported in the blood as phosphate esters 3. . Less than 5% of typhoid cases become chronic carriers 58-Carbon dioxide: 1. A Ramstedt's pyloromyotomy involves splitting of the muscle layers. not bilious. Vomiting normally commences at 2-3 weeks of age. On clinical examination during a feed. 60-The following are recognised causes of drug -induced immune thrombocytopenia: 1. Is transported in the blood in simple solution 4. 59-Infantile hypertrophic pyloric stenosis: 1. 2. Does not affect plasma HCO3 concentration 2. It is more common in Europeans than in the Afro-Caribbean or Asian population.g. hypokalaem ic metabolic alkalosis (raised serum bicarbonate levels) due to loss of gastric acid. postgastrectomy. Results in biochemical changes including low serum chloride. followed by surgery. Treatment is corre ction of the acidbase disturbance. sickle cell disease and those with reduced gastric acidity / motility e.Hydrolyzed to carbonic acid in the red cells.As carbamino-coumpounds in combination with Hb and plasma proteins.Dissolved in simple solution in plasma and RBC. 5.4 kPa during sleep.PEDIATRICS EXAMINATION REVIEW 5. an olive shaped mass can be felt in the right hypochondrium. The low PiO2 at altitude causes hyperventilation. 4. Typical biochemical findings are that of a hypochloraemic. Presents with projectile. Arterial CO2 rises when living at an altitude of 9000 met ers Teaching Notes for Question 58 Theme: Carbon dioxide 1. CO2 is transported in blood in a variety of ways: . Is more common in male babies than female babies 2. elderly. 3. Salmonellae are highly invasive but most infections are mild and self limiting. Quinine 3. With typhoid 15-20% become chronic carriers Teaching Notes for Question 57 Theme: Salmonella infections The risk of infection following ingestion of contaminated food or water is higher in the immunosuppressed. Arterial CO2 rises during sleep 5. which causes the lowering of the PaCO2. and is of feed.

Patients present with acute purpura and platelets often less than 10x10 9/l. 3. All the above drugs can cause thrombocytopenia. Heparin Teaching Notes for Question60 Theme: Drug -induced immune thrombocytopenia. Mannose binding protein deficiency is a known risk factor for meningococcaemia 5. 5. Pigmented 'muddy brown' granular casts suggest acute tubular necrosis. greater extent of proteinuria suggests glomerular pathology. 61-The following statements are correct regarding ur inary analysis in acute renal failure: 1.cent. Frequently co-presents with meningitis. penicillamine and phenylbutazone cause this by bone marrow suppression. Teaching Notes for Question 61 Theme: Acute renal failure Pigmented 'muddy brown' granular casts are c haracteristic of acute tubular necrosis (ATN) and depict the sloughed out tubular epithelial cells. Hyaline casts (inactive/bland urinary sediment) are formed in concentrated urine (of any cause) by the Tamm -Horsfall protein that is secreted by the epithe lial cells of the loop of Henle. Presents in seasonal epidemics 3. RBC casts ('active' urinary sediment) are characteristic of glomerulonephritis of any aetiology. Transparent hyaline casts are nonspecific and may be present in any case of ARF unrelated to the specific aetiology. terminal complement deficiencies (C6 -C9). 62-Concerning meningococcal disease: 1. 2.PEDIATRICS EXAMINATION REVIEW 4. 2. Heparin also causes thrombocytopenia through platelet aggregation. Proteinuria of up to 1 gm per day is common in ATN. Rifampicin 5. 65 . making LP necessary at presentation 4. Ciprofloxacin is used as an alternative prophylactic agent to rifampicin especially in pregnancy or those taking the oral contraceptive pill. The others produce an antibody-drug-plasma protein complex which deposits on the platelet surface leading to either lysis by complement or removal by reticuloendothelial cells. 63-Features of the systemic inflammatory response syndrome (SIRS) include: 1. Is readily distinguished on clinical grounds from other infectious agents by the spreading purpura 2. Respiratory ra te >16/min. There is a good correlation between functional renal impairment and the findings on urinalysis. Core temperature <35 deg. However chloramphenicol. Ciprofloxacin is used for routine contact prophylaxis Teaching Notes for Question62 Theme: Meningococcal disease Other risk factors include splenectomy. RBC casts suggest primarily renal arteriolar diseases such as vasculitis. 4. Presence of proteinuria argues against acute tubular necrosis and suggests a primary gomerular pathology.

the most common pathologic correlate. 65-The following conditions will present with cyanosis in the first week of life: 1. Is uniquely associated with GAD antibodies. 2. Similar antibodies may be found in temporal lobe epilepsy and in diabetes. The spontaneous muscle activity in SMS is of central origin – not peripheral nerve dysfunction . The stiff-man syndrome (SMS) is an uncommon neurological disease. Teaching Notes for Question 63 Theme: Systemic inflammatory response syndrome. then the inhibitory neurotransmitter GABA is decreased and muscles become continuously stimulated by motor neurons. The autoim mune aetiology is confirmed by the finding of insulin-dependent diabetes in 40% of patients. If GAD function is significantly decreased. including diabetes mellitus and seizures. Sepsis is defined a s the development of a systemic inflammatory response syndrome. have been associated with a wide range of human disease.and is related to release of polysynaptic spinal and brainstem reflexes from inhibition. Tachycardia >90 bpm. 5. anti –glutamic acid decarboxylase (GAD) antibodies. This may cause progression to multi-organ dysfunction syndrome. 4. Benzodiazepines and baclofen are also helpful. Teaching Notes for Question 64 Theme: Stiff-man syndrome. Is a predominantly autoimmune syndrome. Hypoplastic left heart syndrome 4. Aortic stenosis 2. over -riding of the aorta and a VSD. 5. Stiff-man syndrome can be treated with steroids and other immunosuppressants. 3. Hypercapnia with PCO2 >6 kPa. Any cardiac lesion which allows a mixing of blood along with a right to left flow or any cardiac lesion wherein pulmonary perfusion is impaired results in cyanosis. Fallot's pe ntalogy includes an ASD along with the tetrad of infundibular pulmonary stenosis.PEDIATRICS EXAMINATION REVIEW 3. Is associated with fibrillation and myokimeia. Fallot's pentalogy Teaching Notes for Question 65 Theme: Cyanosis in the first week . 64-The stiff-man syndrome :1. as it typically will in the first days of life. To further confuse matters. RVH.Babies with tetralogy of Fallot usually have a patent ductus arteriosus at birth that provides additional pulmonary blood flow. so sev ere cyanosis is rare early after birth. cyanosis can 66 . Metabolic alkalosis. 4. Transposition of the great vessels 3. Left heart problems or outflow tract obstructions present as cardiac failure. Is best treated by benzodiazepenes and baclofen. Is a syndrome associated with chronic inflammation of peripheral nerves. The cause in many cases is the presence of antibodies to glutamic acid decarboxylase (GAD). Fallot's tetralogy 5. As the ductus arteriosus closes. characterised by symmetrical rigidity and spasm of predominantly axial and proximal limb muscles. but plasmapheresis or intravenous immunoglobulins are more effective. It may involve one limb only (stiff leg syndrome).

Secondary alcohol . 66-Sturge-Weber syndrome :1.Inflammation In sideroblastic anemia there is Fe. Typically have slit like ventricles on CT 5. Refractory focal tonic-clonic fits lead to a progressive hemiparesis contralaterally. c) Lead poisoning d) Beta-thalassemia (major or intermedia) e) Alpha-thalassemia f) Anemia of chronic disease g) Copper deficiency Sickle cell disease does not produce a microcytic anemia unless associated with thalassemia as in HbSb0. Skull radiograph shows 'pepperpot' skull Teaching Notes for Question 66 Theme: Sturge-Weber syndrome Sturge-Weber is a rare (1 in 50000) sporadic combination of facial naevus and fits. Sickle cell anemia 3.4 If DF is +ve = Fe deficiency If DF is -ve = beta-thalassemia trait 67 .(5 x Hb[g/dl]) . Treatment is with pyridoxine (vitamin B6). calcification and cerebral atrophy. Infantile glaucoma (bupthalmos) commonly occurs. Hypochromic microcytic anemia is usually associated with: a) Fe deficiency b) Sideroblastic anemia: . Occurs in 50% of children with port wine stains in the ophthalmic division of the trigeminal nerve 2.PEDIATRICS EXAMINATION REVIEW develop or become more severe. Folate deficiency 5. hemiparesis and often low IQ. Autoimmune hemolytic anemia 4. Develop hemiplegia on the ipsilateral side of the vascular lesion 3. Alpha thalassemia 2.Malignancy . It occurs in 8% with unilateral lesion and 33% with bilateral lesion. Are associated with cataracts 4. The degree of cyanosis is proportional to lung blood flow and thus depends upon the degree of narrowing of the outflow tract to the pulmonary arteries. 67-Hypochromic microcytic anaemia is seen in: 1. ipsilaterally.3. Diagnosis is by MRI. Imerslund syndrome Teaching Notes for Question 67 Theme: Hypochromic microcytic a nemia. CT shows unilateral dilated ventricle. To differentiate between Fe deficiency and beta -thalassemia use: 'Discriminant function'(DF) = MCV(fl) . T he skull radiograph shows the intracranial calcification in the occipitoparietal lesions as ‘tram -line’ or ‘railroad track’ calcification.RBC (x109/l) .Primary X-linked recessive . but accumulation in the RBC mitochondria results in stippling on the peripheral smear and ring sideroblast formation in the bone marrow.

Autoimmune disease occurs in about 10% of cases. rather than absence seizures likely. IgM levels is also reduced in 50% of patients. Immunological findings are variable. Granulomatous lesions have been reported in lymphoid tissues. this is no use in hemodilution states (i. Autoimmune disease 3. include: Later onset. viruses or Candida. A normal EEG Teaching Notes for Question 69 Theme: Childhood seizures Complex partial seizures may present with brief periods of reduced awareness. and in some cases susceptibility to infections with Pneumocystis carinii. patients have marked reduction in serum IgG and IgA. haemolytic anaemia or SLE. Inflammatory bowel disease 2. 69-A 9 yr old girl is seen in clinic. A good response to anti-epileptic drugs 5. Imerslund syndrome is characterized by: a) Chronic mucocutaneous candidiasis b) Decreased PTH c) Proteinuria d) Decreased vitamin B12 absorption. 68-Which of the following is associated with common variable immunodeficiency? 1. but it is the humora l immune deficits that usually present clinically.PEDIATRICS EXAMINATION REVIEW Beware. Reporting unusual smells prior to the episodes 2. Patients present with recurrent pyogenic infections. and skin and share many clinical properties typical of sarcoidosis. solid organs. Features which make complex partial seizures. longer duration. focal 68 .(CVI) CVID refers to a spectrum of disorders in which cell -mediated and antibody deficiency are combined in variable amounts. Granulomatous lesions 5. Her teacher at school has reported that she has numerous episodes throughout the day of abruptly staring into space mid -conversation for a few seconds with rapid return of awareness. Abnormal features on MRI scanning of the brain 4. Megaloblastic anemia on the other hand is usually due to: a) Folate deficiency b) Vitamin B12 deficiency c) Orotic aciduria d) Pyruvate kinase deficiency To discriminate between folate and B12 deficiency .think: -Decreased RBC folate -Hyper-segmented neutrophils -LDH All suggest folate deficiency. In CVID.e. Susceptibility to recurrent parasitic infections Teaching Notes for Question 68 Theme: Common variable immunodeficiency . Some T -cell functional assays may be abnormal. Normal levels of IgA 4. usually immune thrombocytopenia. Which of the following features suggest complex partial rather than absence seizures as the cause of her problem : 1. There is an increased incidence of malignancy. the presence of aura and post -ictal phenomena. post hemorrhage or in pregnancy). Granulomas are a special feature of CVID and do not occur in other primary lymphocyte disorders. Provocation of the episodes by hyperventilation 3.

g. G -CSF is commonly used to treat neutropaenic sepsis. Peak flow monitoring is an easy way to monitor some aspects of lung function and can be used in most children >5 years and in some who are younger. 70-The following statements are reagrding asthma: 1. The first manag ement step would be :1.g. IFN-gamma is used to improve macrophage function in patients with chronic granulomatous disease. At low concentration. It is produced mainly by activated mononuclear phagocytes. At high concentrations. Chemokines are chemoattractant cytokines and recruit immune and other haemopoietic cells to sites of inflammation Cytokines are increasingly used in the therapeutic arena e. bronchodilators and glucocorticoi ds respectively. but are not a first line treatment. IFN -gamma is produces by activated CD4+. epithelial cells and endothelial cells. Inhaled glucocorticoids improve lung function in asthmatics 5. it causes pyrexia and enhances acute phase protein synthesis. Temp 39. Can be used clinically to help treat infection 5. Glucocorticoids can improve lung function with regular use. IFN -alpha is used as an anti-viral agent. for example) and a poor response to anti epileptic therapies. Chemokines can induce the transmigration of T cells into inflamed tissues 4. 72-15-year-old teenager presented with few haehorrhagic spots. but the two main areas remain those of symptom relief and those that prevent attacks.PEDIATRICS EXAMINATION REVIEW abnormalities or normal EEG (a bsences tend to have typical 3HZ spike pattern). agitation and confusion having been to school. CD8+ and NK cells. but is also secreted by other cells e. Peak flow monitoring can be used in most children aged 5 years and above Teaching Notes for Question 70 Theme: Asthma Asthma remains a c linical diagnosis without a highly specific or sensitive test. The diagnosis of asthma involves blood tests 3. pulse 110 and BP 120/80. IL-1 causes pyrexia and induces protein synthesis in the liver 2. IL1 mediates local inflammation. associated MRI abnormalities (mesial temporal sclerosis. Blood tests are not usually helpful. Treatments for asthma continue to evolve. and IFN-beta is used to treat multiple sclerosis. Oxygen 2. Interferon gamma causes increased expression of MHC class II molecules on antigen presenting cells 3. The most reliable investigations are those of lung function that show reversibility of bronchospasm. Treatment can be divided into those that prevent and those that relieve symptoms 4. 71-Regarding cytokines:1. Leukotriene inhibitors have a useful role in some patients. Leukotriene antagonists are a fi rst line treatment in asthma 2. It is a potent activator of macrophages and promotes the differentiation of Th1 cells. IL-2 s the main cytokine produced by naïve T cells Teaching Notes for Question 71 Theme: Cytokines IL1 is known as endogenous pyrogen. Intravenous fluid 69 .

or when complex anatomic defects. Teaching Notes for Question 74 Theme: Hypoadrenalism Addison's disease leads to hypoadrenalism. In addition. 4. Intravenous antibiotic 5. Amyloidosis. 74-Causes of hypoadrenalism include: 1. Total anomalous pulmonary venous drainage (TAPVD) 3. 73-The following congenital lesions are associated with cyanosis: 1. Coarctation of the aorta 4. 3. unassociated with RV outflow obstruction.PEDIATRICS EXAMINATION REVIEW 3. 2. 5. Throat swab 4. 75-In Wiskott-Aldrich syndrome: 1. cyanosis may be caused by persistence of fetal pathways. Aortopulmonary window 2. Tuberculosis. Inheritance is autosomal recess ive 70 . The aortopulmonary window defect is a communication between the ascending aorta and the main pulmonary artery. cause mixing of the pulmonary and systemic venous return. The presence of pulmonary and aortic valves and an intact ventricular septum distinguishes this anomaly from truncus arteriosus. Cushing's disease. Metastatic cancer. There is an increased risk of malignancy 4. IV antibiotics must be given immedietly. also called as Waterhouse Friederichsen disease. Lumbar puncture Teaching Notes for Question 72 Theme: Systemic Sepsis The history suggets meningococcal sepsis. The immunodeficiency is restr icted to T cells only 2. Tetralogy of Fallot 5. Cushing's disease requires adrenalectomy for hyperfunction. which follows meningococcal septicaemia. Eisenmenger's syndrome Teaching Notes for Question 73 Theme: Cyanotic heart disease Congenital heart disease produces cyanosis when obstructio to RV outflow causes intracardiac right-to-left shunting. IgA and IgE levels are often low 5. Platelets are of normal size. but are few in number 3. Severe sepsis. Severe sepsis can cause haemorrhage in adrenals.

Immunodeficiency. bradycardia.which plays a role in cytoskeletal organisation and possibly intracellular signalling. Malignancy. There is an increased risk of lymphoid malignancy. 77-The following tumour markers are strongly associated with the tumours shown: 1. and also sensitive. CA-19. CA-125 and pancreatic carcinoma.9 and ovarian carcinoma. * The classical features are lethargy. Teaching Notes for Question 77 Theme: Tumour markers The ideal tumour marker would be specific to a particular tumour. always being detectable if the tumour was present and present at a concentration directly propor tional to the volume of disease present. 71 . The defect is due to a deficiency in the Wiscott–Aldrich protein. As no tumour marker fulfils these ideals.9 with pancreatic cancer. Bradycardia 2.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 75 Theme: Wiskott-Aldrich syndrome Wiskott-Aldrich syndrome is an X-linked combined immunodeficiency. CEA is generally associated with colorectal cancer (also may be increased in other GI malignancies and in smokers). CEA and colorectal carcinoma. 3. and they are usually more useful for monitoring response to treatment or disease recurrence than primary diagnosis. Eczema). 76-The following are consistent with myxedema: 1. Beta-HCG and choriocarcinoma.Bone marrow transplant is curative. Microvilli on T-cells appear abnormal. Platelets are not only few in number but are also small and these children can present w ith life-threatening bleeding. normal levels of IgG and high levels of IgA and IgE. Beta-HCG is raised in hydatidiform mole and choriocarcinoma and testicular tumours (esp. * Drug metabolism and excretion are reduced. As well as impaired cell mediated immunity (with lymphopenia) there are low levels of IgM. so these p atients often exhibit increased sensitivity to certain drugs including opioids. teratoma). Pneumocystis carinii and herpes simplex virus. 2. Cardinal features are thrombocytopenia. hoarse voice. 4. (mnemonic TIME: Thrombocytopenia. Antibody responses to polysaccharide antigens are deficient. Alpha-FP and hepatocellular carcinoma.g. Patients also have recurrent respiratory infections and are susceptible to infections with organisms e. CA-125 is associated with ovarian and CA-19. Decreased sensitivity to opioids Teaching Notes for Question 76 Theme: Myxedema * Hypothyroidism (myxedema) can be due either to pituitary disease or autoimmine thyroiditis (Hashimoto's disease). weight gain. delayed deep tendon reflexes. compromise must be sought. intolerance to cold. Intolerance to heat 3. greasy skin and a proximal myopathy. undetectable if the tumour was not present. Myopathy 5. Dry skin 4. seminoma). eczema and immunodeficiency. Alpha-FP is increased in hepatocellular carcinoma and in testicular germline tumours (esp. 5.

72 . These episodes last hours to days. C4 levels tend to be low during an attack 5.Salbutamol causes bronchodilation and increases the PEFR and FEV1. Deficiency of C1 esterase inhibitor also results in increased formation of bradykinin which enhances oedema by acting on vascular endothelium. Can cause an increase in PEFR 2. Atrial fibrillation 3. This glycoprotein regulates the clas sical complement pathway by dissociating C1r2s2 from C1q. Stress. Attacks can be triggered by exercise 4. Has no effect on hypoxic-pulmonary vasoconstriction 4. Clinical features include recurrent episodes of oedema of skin. gut causing abdominal pain. Danazol is used for treatment Teaching Notes for Question 80 Theme: Hereditary angioedema Features of hereditary angioedema: Autosomal dominant Due to a deficiency of C1 esterase inhibitor.It interferes with this mechanism (hypoxic pulmonary vasoconstriction) hence an adequate FiO2 is required during its admin. May cause a rise in insulin release 5. Protein C deficiency 5. Has no effect on the FEV1 3. atrial fibrillation and polycythaemia. In its absence.PEDIATRICS EXAMINATION REVIEW 78-Arterial thromboses are associated with: 1. surgery or exercise can trigger attacks. Polycythaemia Teaching Notes for Question 78 Theme: Arterial thromboses Arterial thromboses may be associated wi th Behcets syndrome. 79-Salbutamol: 1. It can present with abdominal pain and vomiting 3.It causes a shift of potassium ions into cells. 80-Regarding hereditary angioedema: 1.2 . 3 . hence the rise in insulin.diarrhoea and vomiting and the upper airway. there is increased consumption of complement components C2 and C4 with the production of fragments that mediate oedema. 4. It may also increase the plasma concentration of free fatty acids and glucose. Factor V Leiden mutation 4.5 . Causes hypokalemia after prolonged use Teaching Notes for Question 79 Theme: Beta agonists 1. It is due to deficiency of complement component C1 2. Factor V Leiden mutation and protein C deficiency are associated with venous thromboses. Behcets syndrome 2.

4: Asymmetrical bre ast development is a common phenomenon and just needs reassurance. Boys' genital development is described by 5 stages 2. Asymmetrical breast development is common in girls during puberty 5. Testes/scrotum darken adult * Female: Breasts Stage I prepubertal II III IV V breast bud breast and areola enlarge breast and areola enlarge to form mound above main breast adult . downy hair III IV V darker. 5: A recent BMJ paper looked at a sample of 100 girls to establish the average age of menarche. 82-Hereditary angioedema is:1. Pubic hair in the prepubertal state is described as Stage 0 4. Breast development stage 3 refers to breast buds 3.PEDIATRICS EXAMINATION REVIEW Treatment is with either danazol (boosts C1 esterase inhibitor levels) or tranexamic acid (inhibits plasmin.8 years). There was no difference related to social class or ethnicity. Inherited in an autosomal recessive fashion 73 . No data has been collected in a UK population since the 1950s. curlier and spreading adult type hair but not on medial thighs yet adult * Male: Genitals Stage I II III IV V prepubertal scrotum and testes grow and scrotal skin coarsens penis lengthens and testes grow penis broadens and glans develops. * Pubic Hair: Stage I prepubertal II sparse. Menarche is occurring in increasingly younger girls over the past three decades Teaching Notes for Question 81 Theme: Stages of puberty James Tanner devised stages in the 60s to describe male puberty by pubic hair and genital development and female puberty by pubic hair and breast development (as well as by menarche).only nipple projects * Menarche is either 0 or 1. The median age of menarche was 12 years and 11 months. The much talked about drop was from late 19th century (14. 81-Regarding Tanner stages of puberty: 1. This suggests that there has been no decrease in the average menarcheal age in the past 3 decades.75 years) to 1950s (12. which consu mes C1 esterase inhibitor).

The inhibitor of C1 is grossly lacking and the resulting acute. Marfan's syndrome 2. Plasmin mediates the release of C2 kinin and therefore is amenable to blockade with e-aminocaproic acid as an alternative first line therapy. Upper airway obstruction 4. Purified C1 inhibitor can be given in acute severe episodes and should be given pre -operatively should one ever be required. Pia is responsible for production of CSF Teaching Notes for Question 83 Theme: Meninges * The dura's rich arterial system supplie s the bone and dura. as it induces acne. This however. is far from popular with the girls. It causes swelling (spontaneously or following trauma) of the following: i) skin -especially the face ii) intestine . Treated with regular C1 inhibitor infusions Teaching Notes for Question 82 Theme: Hereditary angioedema Hereditary angioedema is an autosomal dominant condition. * Dural stimulation is very stimulating. weight gain and amenorrhoea. Arachnoid folds project inwards to form the falx cerebri and falx cerebelli 4. rickets or upper/lower airway obstruction. circumscribed and non inflammatory oedema is mediated by the production of vasoactive C2 kinin. The dura is adherent to the bone 2. A consequence of C2 kinin production 3. vomiting & diarrhoea but no point tenderness iii) airway . 83-Regarding the meninges: 1. 84-Pectus excavatum is associated with: 1. it may be a manifestation of Marfan’s . Rightward shift of the heart Teaching Notes for Question 84 Theme: Pectus excavatum Midline narrowing of the thoracic cavity (pectus excavatum) is usually an isolated finding. subdurals follow the form of the cerebral hemispheres. * Extradural hematomas form a lentiform shape. choking and is potentially fatal. but it may be 74 . * The dura project inwards to form the falx.causing laryngeal oedema. Sometimes treated with the anabolic steroid danazol 5. Pia stimulation during surgery is very stimulating 5. A cause of spontaneous life-threatening laryngeal oedema 4. Rickets 3. Subdural hematomas form a lentiform shape 3. As the patients are heterozygotes they will produce very small amounts of C1 inhibitor. Exercise limitation 5.resulting in abdominal pain. A link has been suggested between pectus excavatum and exercise limitation.PEDIATRICS EXAMINATION REVIEW 2. which can be stimulated with regular danazol (an anabolic steroid). But. and depth of anesthesia may need to be alter ed in response.

85-Renal ultrasound: 1. Although more common in teenagers. Recommended when CD4 count drops below 1000microlitre 5. but also indicate the large amount of operator dependence found with all USS examinations 86-Regarding P. Whereas renal USS is good for detecting distal ureteric dilation. The broad ranges given relate to various studies. Has a false negative result of 10 . CD4 count less than the cut off for the age or less than 200 in all ages.45% when looking for renal scarring. Is a reliable means of looking for vesico ureteric reflux (VUR). Intravenous Pentamidine is recommended 3. Has a false negative result of 15 . Androgens promote seborrhoea. 5. Has a false positive rate of 25 . Is a good means of detecting distal ureteric dilation. but most females have normal levels of androgens . Oral candidiasis in HIV patients warrants Prophylaxis Teaching Notes for Question 86 Theme: Pneumocystis carinii prophylaxis Primary prophylaxis is warranted for all infants born to HIV mothers until infection is ruled out in the infant.50% when looking for VUR.acnes) and (4) Inflammation. Dapsone has also been tried.Carinii prophylaxis: 1.50%). persistant oral candidiasis and progressive weight loss. 3. it is poor at detecting reflux (the false negative rate is 25 .their sebaceous 75 . It is also poor at detecting scarring. the heart is shifted leftward. MVP and WPW syndrome appear to be ot her abnormalities associated with pectus excavatum. Oral steroids are a recognised cause 3. It is recommended in all infants born to HIV infected mothers 2. Drugs used are cotrimo xazole and aerosolized pentamidine.PEDIATRICS EXAMINATION REVIEW habitual related to parental fears. as this is often not present at the time of scanning. Females with acne commonly have elevated blood androgens 2. 2. 4. Teaching Notes for Question 85 Theme: Renal ultrasound scan. Previous P. Interleukin 1 alpha promotes comedogenesis 4. In many children. 87-The following are true about acne:1. Carinii infection warrants prophylaxis 4.20% when looking for renal scarring. 7% of in dividuals have acne until age 40. Oral isotretinoin causes hyperlipidaemia 5. The enzyme Type 1 5 alpha reductase is expressed in sebaceous gland ducts Teaching Notes for Question 87 Theme: Acne The four components of acne are (1) Seborrhoea (2) Comedogenesis (3) Colonisation of the duct with Propionibacterium acnes (P.

It is a teratogen. and drugs such as oral steroids and anabolic steroids. 5. hair thinning. Fixed. Increases calcium and dec reases phosphate reabsorption in kidney 2. The overall effect is to increase calcium and phosphate level in the blood Teaching Notes for Question 89 Theme: Parathyroid The overall effect is to increase calcium amd decrease phosphate level in the blood. Increases osteoclastic activity 3. 4. IL-1 alpha promotes comedogenesis. Absent gag reflex with pharyngeal stimulation.g. Apart from physiological acne. doxycycline. muscle aches. is very rare (incidence less than 1 in 10000) 3. minocycline. Activity of this enzyme is high in sebaceous glands of acne -prone individuals. Neuromuscular drugs should have been stopped for at least twelve hours. May present with autoimmune disease 4. 90-Selective IgA deficiency:1. 89-The following are true regarding parathyroid hormone actions: 1. acne occurs in pathological conditions of congenital adrenal hyperplasia. Other side effects: headaches. dilated pupils unresponsive to light. Type 1 5 alpha reductase is an enzyme converts testosterone to dihydrotestosterone which binds to receptors in sebaceous glands increasing sebum production. No motor response from painful stimuli. 3. Is treated with iv sandoglobulin 2. dry lips and skin. Treatment: Progress stepwise from (1) Topical treatments (2) Oral tetracyclines (3) Second-line antibiotics e. Increases 1. Oral isotretinoin can cause hyperlipidaemia and der anged liver chemistry. Decreases calcium and increases phosphate reabsorption in kidney 5. Comedones represent hyperproliferation of the sebaceous follicles. mood alterations. 2. 88-The following criteria must exist before a diagnosis of brain death can be made: 1. trimethoprim (4) Dianette (cyproterone acetate and ethinyloestradiol) in women (5) Isotretinoin. IgG2 subclass deficiency may also be present with more severe infective complications 5. No eye movement when 20 mls cold water is perfused into the external auditory meatus.25 dihyroxy vitamin D3 production in the kidney 4. dry eyes. Teaching Notes for Question 88 Theme: Brain death An absent gag reflex must be assessed with bronchial stimulation via a tracheal catheter. erythromycin.PEDIATRICS EXAMINATION REVIEW glands respond excessively to normal levels of androgens (end -organ hypersensitivity). Neuromuscular drugs should have been stopped for at least 24 hours before assesment can be made. is associated with HLA-DR3 76 .

secreting plasma cells. Alcohol also reduces the secretion of oxytocin. Exercise. Has anti-sialogogue properties 4. clofibrate. * Alcohol. exercise and standing. If present. The defect in this syndrome is a block in differentiation of IgA-expressing B cells to antibody . 5. making it a good agent to be used in asthmatics. Vomiting. 93-Sickle cell disease :- 77 . It is usually sporadic. Autoimmune syndromes (e. O2 consumption and ICP and frequently leads to emergence phenomena. Treatment is symptomatic – antibiotics for infections 91-The following increase vasopressin secretion: 1. either for induction or as a 3rd line treatment of an acute attack in a ventilated patient. * Nausea and vomiting. Is a potent respiratory depressant Teaching Notes for Question 92 Theme: Intravenous anesthetic agents * Ketamine is a mild respiratory stimulant and a potent bronchodilator. * Decreased ECF volume. Is more emetogenic than thiopentone 3. rheumatoid arthritis . Some patients may have additional IgG subclass deficiencies. * Pain. * It causes more post-op nausea and vomit ing than thio and propfol (but less than etomidate) and cholinergic stimulation leads to increased saliva production. * It causes increased cerebral blood flow. but can be inherited in an autosomal dominant or autosomal recessive pattern. Autoantibodies (organ -specific and non-specific) are often found even in the absence of clinical disease. stress. Haemorrhage. 2. Morphine. 3. Teaching Notes for Question 91 Theme: Vasopressin The following stimulate vasopressin secretion: * Increased plasma osmolality. Sjogrens disease. The following decrease vasopressin secretion: * Decreased plasma osmolality. morphine and carbamazepine. pernicious anaemia. May be used safely in patients with suspected raised intra -cranial pressure 5. 92-Regarding ketamine: 1. DR3 and deletions of the C4A gene. * Angiotensin II. thyroiditis) are associated . Alcohol.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 90 Theme: Selective IgA deficiency Selective IgA Deficiency is the commonest immune deficiency (affects 1 in 500 -700 people). clinical features are usually mild – recurrent sinusitis and respiratory infections (mild) or chronic diarrhoea. There is an association with MHC alleles HLA B8. * Increased ECF volume. Serum IgA levels are usually < 50µg/ml but serum IgG and IgM levels usually normal. Anti -IgA antibodies occur in 30 -40% and these may cause anaphylaxis if transfusi ons are administered.g. Patients are often asymptomatic. 4. Is contra -indicated in reversible airways obstruction 2.

Is a direct continuation of the inte rnal iliac 3. Radiofemoral delay is the diagnostic sign 78 .Profunda femoris . which detects the presence of sickle haemoglobin.Highest genicular 95-Coarctation of the aorta:1. The branches of the femoral artery are: . Can be co-inherited with alpha or beta -thalassaemia Teaching Notes for Question 93 Theme: Sickle cell disease Sickle cell disease is an autosomal recessive condition caused by a single amino acid mutation in the Beta globin gene on chromosome 11.Deep external pudendal .Superficial external pudendal . Patients with se vere clinical manifestations of sickle disease have been treated with allogeneic bone marrow transplantation from an HLA -identical sibling. especially in the neonatal period. occuring in 25% of patients with homozygous sickle disease by the age of 45 years. is often not positive until 6-9 months of age and cannot be guaranteed until one year of age. Autologous bone marrow transplantation is where the patients own bone marrow is replaced and would not be of benefit in this condition. Runs as a single trunk from the inguinal ligament to the lower border of the popliteus 2. lies in the adductor canal (Hunter’s canal) 5.Superficial epigastric .Superficial iliac circumflex .Muscular . 5. This is why the sickle test. In the upper third of the thigh the femoral artery is contained in the femoral triangle (Scarpa’s triangle) 4. Will give a positive sickle test result at birth 3. The superficial epigastric artery is a branch of this vessel Teaching Notes for Question 94 Theme: Vascular anatomy . Is an indication for autologous haemopoietic stem cell transplant 5. Clinical effects do not become apparent until 6 to 12 months of age when haemoglobin switching occurs and the amount of HbF (fetal haemoglobin) decreases and the amount of HbS (sickle haemoglobin) increases. In the lower third of the thigh. The alpha globin genes are coded on chromosome 16. Renal failure may arise. Clinical problems include strokes and these are common.femoral artery The femoral artery is a direct continuation of the external iliac artery. Is associated with notching of the anterior ribs 4. Is associated with strokes in 25% of patients by the age of 45 years 4. Is typical of Marfan’s Syndrome 2. 94-The femoral artery: 1. It can be co-inherited with alpha or beta thalassaemia.PEDIATRICS EXAMINATION REVIEW 1. Is caused by a mutation on chromosome 16 2. Is typical of Noonan syndrome 3.

Notching of the inferior border of the ribs from pre ssure erosion by enlarged collateral vessels is common by late childhood seen in posterior one third of the ribs. pain localising close to the umbilicus 3. * Pain. vomiting. It is a feature of Turner’s syndrome and is associated with a bicuspid aortic valve in more than 70% of patients.it is metabolised by the liver and kidneys. exercise. presentation at age 10 2. * Activation of V2 receptors in the distal tubule and collecting duct leads to increased water permeability via cAMP . 97-In recurrent abdominal pain in children. altered bowel habit. alert you to this possibility. sleep and morphine all increase ADH as does a rise in the osmolality of the extracellular fluid and hemorrhage. * It acts on V1 receptors in the vascular smooth muscle giving it vasoconstrictor properties. Has a half-life in the plasma of approximately 24 hours 3. family history of migraine 4. pain during daytime only 5. the following suggest an u nderlying organic cause: 1. but 98% occur just distal to the origin of the left subclavian artery at the origin of the ductus arteriosus. Certain features should. Secretion can be suppressed by morphine 5. This variant most typically occurs in children. Obviously. however. having a half -life of 5 minutes in vivo . Mitral valve abnormalities (a supravalvular ring or prolapse) and subaortic stenosis are associated lesions. 96-Anti-diuretic hormone (ADH): 1. *Alcohol is a well-known inhibitor of ADH. PR bleeding and constitutional symptoms are all worrying signs. stress. Is a decapeptide 2. vomiting Teaching Notes for Question 97 Theme: Recurrent abdominal pain. Abdominal migraine is one of the variants of migraine headache. They usually have a family history of migraine and go on to develop typical migraine later in their life. This group of left -sided obstructive lesion occurring together is termed the Shone complex. the more likely the presence of an organic cause. dysuria. Radiofemoral delay is due to the blood flow to the descending aorta being dependent on collaterals. and non -organic causes can only be suggested after the exclusion of the organic ones! 79 . These include presentation at less than 4 or over 15 years and pain that wakes the child at night. The anomaly is commoner in males (2:1. It is also known by other terms including 'periodic syndrome'. M:F). Secretion is increased by pain Teaching Notes for Question 96 Theme: Anti -diuretic hormone * ADH (vasopressin) is a nonapeptide. It is said that the further the pain is from the umbilicus. Acts on the distal tubule of the kidney to increase reabsorption of water 4.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 95 Theme: Coarctation of the aorta Constrictions of the aorta of varying degrees may occur at any point from the arch to the iliac bifurcation. Recurrent abdominal pain is very common and seldom has an organic cause.

Its half -life is 14 hours and its main function is to reduce the surface tension of the alveoli. Is partly recycled by endocytosis into the synthesizing cell 2. * Basal cell carcinoma is an invasive condition. Xeroderma pigmentosum 2. Hereditary spherocytosis 3. Synthesis is stimulated by glucocorticoids Teaching Notes for Question 100 Theme: Surfactant Dipalmityl-phosphotidyl choline is the mai n component of surfactant and is produced by Type-11 alveolar cells (granular pneumocytes). 100-Pulmonary surfactant 1. 99-The following skin conditions are by definition associated with epithelial dysplasia: 1. G6PD deficiency is X -linked recessive. * Seborrhoeic keratosis is a common skin lesion. * Xeroderma pigmentosum is a condition of defective DNA repair. Seborrhoeic keratosis Teaching Notes for Question 99 Theme: Dysplasia in the skin. Is produced by type 1 alveolar cells 3. Basal cell carcinoma 4. Reduction in pulmonary flow can cause a decrease in surfactant production 4. Hereditary spherocytosis is a dominantly inherited disorder with variable expression. which is not associated with dysplasia. 101-Regarding sleep disturbance: 80 . Congenital adrenal hyperplasia 4. Synthesis is inhibited by thyroxine 5. Actinic keratosis 5. Although later on in the condition dysplastic lesions such as actinic keratosis may occur. Bowen’s disease of skin 3. Congenital adrenal hyperplasia and galactosaemia are inherited in an autosomal recessive manner. Neurofibromatosis type I 2. G6PD deficiency Teaching Notes for Question 98 Theme: Autosomal dominant inheritance Neurofibromatosis type I is inherited as an autosomal dominant disorder. * Actinic keratosis is a condition in which epidermal dysplasia is induced by sunlight. * Bowen’s disease of the skin is by definition associated with carcinoma in situ of the epidermis. at least in the early stages. the condition itself is not necessarily associated with dysplastic changes.PEDIATRICS EXAMINATION REVIEW 98-The following are inherited in an autosomal dominant manner 1. Galactosaemia 5. There is however a high rate of spontaneous mutation and approximately 50% of affected individuals represent new mutations.

can increase transcription of the iNOS gene. stimulation of these cells with cytokines or growth factors. 5. of which the individual has very little recall. Increases cAMP. There are three isoforms of nitric oxide synthase (NOS): neuronal NOS (nNOS). Its synthesis is catalysed by enzymes known as nitric oxide synthases (NOS). Is important in the maintenance of pulmonary vascular flow. Sleep terrors are associated with intense vocalisation 5. The biological effects of NO are mediated through its reaction with a number of targets 81 . angiogenesis. 4. 102-Nitric Oxide (NO): 1. producing NO in the process. 3. often rushing to the door as if trying to escape. iNOS activity does not depend on intracellular calcium levels. In most cell types iNOS protein levels are very low or undetectable. Nightmares are dream experiences loaded with anxiety or fear. Oxygen and NADPH are co -factors in this reaction. but like the other NOS enzymes. security. Is critical in increasing endothelin (ET -1) levels. These enzymes convert arginine into citrulline. immune defence and apoptosis etc. neurotransmission. Sleep terrors last at least twenty minutes 4. -Sleep terrors * Occurs in non REM sleep * May be remembered * Considerable autonomic discharge * Appreciable vocalisation * Appreciable body motility * Last no more than 1–10 minutes -Nightmares * Occur in REM sleep * Usually vivid * Some autonomic discharge * No appreciable vocalisation * No appreciable body motility * Can continue into the second half of sleep In a typical sleep terror. Nightmares are associated with appreciable body movements 3. 2. Often there is recurrence of the same or similar frightening nightmare themes. endothelial NOS (eNOS) and inducible NOS (iNOS). and high levels of autonomic discharges. Teaching Notes for Question 102 Theme: Nitric Oxide (NO): Nitric Oxide is a critical molecule involved in processes as diverse as vasodilation. Is the same substance as EDHF. cell signalling. Nitric oxide is a short-lived molecule (with a half-life of a few seconds). All three isoforms can be found in a variety of tissues and cell types. or self esteem. motility. The dream experiences are extremely vivid and usually include themes involving threats to survival. However.PEDIATRICS EXAMINATION REVIEW 1. the binding of calmodulin is essential for iNOS activi ty. nNOS and eNOS are constitutively expressed in mamma lian cells and synthesise NO in response to increases in intracellular calcium and thus calmodulin levels. child sits up or gets up with a panicky scream. Nightmares are associated with intense vocalisation Teaching Notes for Question 101 Theme: Sleep disturbance Sleep terrors or night terrors are nocturnal episodes of extreme terror and pan ic associated with intense vocalisation. with subsequent production of high concentrations of NO. Sleep terrors occur in non -REM sleep 2. Operates by inhibiting PDE’s.

Vitamin B1 deficiency 3. These may be manifest by increased infection rates (e. In addition with acute or chronic respiratory failure (pH less than 7. NO may act as a mediator of inflammatory processes. wound). As part of its plethora of functions. It enhances the effect of cyclooxygenases and stimulates the production of pro -inflammatory eiconosoids. COPD exacerbations are frequently associated with influenza and r hinovirus infection. Trace element deficiencies often go hand in hand with malnourishment.35) should receive antibiotics. there should be given antibiotics. NO is also called endothelium-derived relaxing factor (EDRF) . Nitric oxide acts as a neurotransmitter in the central and peripheral nervous systems. There is a positive correlation in mortality and the number of smoking pack years 4. 104-The following are true of COPD exacerbations: 1. Such a diverse range of potential targets for NO explains the large number of systems that utilize it as a regulatory molecule. Vitamin C deficiency 4. DNA synthesis and mitochondria. it modulates firefly lighting! 103-Factors associated with poor wound healing include: 1. followed by S. NO has been shown to kill cells by disrupting enzymes involved in the Kreb's cycle.it mediates the relaxation of vascular smooth muscle. NO is now known to be the physiological mediator of penile erections.PEDIATRICS EXAMINATION REVIEW such as haem groups. As a consequence of this. Folate deficiency 5. NO modulates endothelin and maintains a low pulmonary vascular resistance. urinary. Moraxhella catarrhalis is the most frequently implicated organism in exacerbation Teaching Notes for Question 104 Theme: Exacerbations of COPD Haemophilus influenzae is the most frequent cause.The following features are associated with increasing mortality with 82 . It is also involved in regulating apoptosis in neurons.35 is an indication for antibiotics according to the BTS guidelines 5. Vitamin A deficiency 2. hypertension and myocardial infarction. The NO formed in vascular endothelium diffuses to smooth muscle cells. All patients should be given antibiotics 2. chest. Zinc deficiency Teaching Notes for Question 103 Theme: Wound healing Patients who are malnourished have poorer outcomes because of impairment and eventual failure of physiological protein -dependent functions. abnormal regulation or control of NO synthesis is capable of affecting a number of important biological processes and has been implicated in a variety of diseases.g. activated macrophages produce high concentrations of NO in response to LPS and interferon -gamma. Thus all patients with moderate or severe disease should be offered influenza vaccination seasonally. According to the BTS guidelines if a patient has two out of the following criteria. Insufficiency is believed to contribute to the pathogenesis of diseases such as atherosclerosis. sulfhydryl groups -to produce nitrosothiols. slo wer wound healing. NO is produced by a number of immune cells. pH less than 7. and activates guanylyl cyclase producing cGMP. In particular. which mediates smooth muscle relaxation.pneumoniae and Moraxhella catarrhalis in causing bacterial exacerbations.and iron and zinc clusters. wound breakdown and dehiscence and death. increased shortness of breath. Importantly. Are associated both with rhinovirus and chlamydia pneumoniae infection 3. in creased sputum volume and increased sputum purulence.

PEDIATRICS EXAMINATION REVIEW COPB exacerbations. amaurosis fugax and retinal arteriolar occlusions all appear to occur more frequently. Decreased in pregnancy 83 . Mitral valve prolapse is a common syndrome and the vast majority of patients are asymptomatic. This is supplied by the right CA (RCA) in 85% cases (‘right dominant circulation’) and by the left circumflex (LCx) in the remaining (‘left dominant circulation’). Lower than intracellular pH 3. The click is often followed by a mid . 107-Normal arterial pH is : 1. The ‘dominant’ coronary artery (CA) is the one that supplies the posterior part of the interventricular septum. Chest pain 4. A variety of non-specific symptoms may be present including palpitations. low FEV1 chronic sputu m production and the number of total pack years. Right coronary artery is dominant in over three -fourth of cases. Bacterial endocarditis 3. cerebellar infarcts. Not essential to maintain ion distribution 4. Acute hemiplegia. A systolic 'click' on auscultation 5. the incidence rises in patients with a systolic murmur. Foetal arrythmias Teaching Notes for Question 106 Theme: Mitral valve prolapse. Most of the venous drainage of the heart occurs via the the Basian system of veins. and when MR is severe. 105-The following statements are correct regarding the cardiac anatomy: 1. chest discomfort. There is an increased risk of infective endocarditis. 4. draining into the coronary sinus and then into the right atrium. The unique physical finding in MVP is a systolic click at least 0. 3. The left atrium is larger in size as compared to the right atrium. a large majority remaining asymptomatic for many years. Teaching Notes for Question 105 Theme: Cardiac anatomy The right atrium is larger in size than the left atrium in normal people. Measured rather than calculated in a pH probe 5. 5. These are greater age. Equal to [H+] of 34-46 nmoles/L 2. postural hypotension. although the incidence appears to be extremely low in patients with a midsystolic click only. symptoms of diminished cardiac reserve predominate. There is one pulmonary vein from each lung that enters the posterior wall of the left atrium.14sec after S1. 106-Mitral valve prolapse is associated with:1. Cyanosis 2. The AV node is supplied by the left anterior descending artery in most of the cases. The outlook for MVP in children is excellent. The sinoatrial node is almost always supplied by the RCA.to latecrescendo systolic murmur that continues to A2. Autonomic dysfunction occurs commonly in MVP and this has been suggested as the cause for the chest discomfort. 2. TIAs. The 4 pulmonary veins drain into the left atrium. Venous drainage from the heart is mainly into epicardial cardiac veins.

CTL kill target cells by Fas ligand-mediated apoptosis as well as by perforin -mediated cytolysis. Laxative abuse and self-induced vomitting may occur in the context of anorexia. brittle hair and nails and dry skin. 109-Defective Fas-FasL interaction leads to impairment of : 1. Examination findings include emaciation. The following factors would be against a diagnosis of an orexia nervosa:1. Recruitment of neutrophils Teaching Notes for Question 109 Theme: Fas .45 = [H+] 36-45nmol/L B. Organophosphates . Helper T-lymphocyte activation 3. It is characterised by an obsessive refusal to eat and altered body image. esp ecially in infancy and results in chronic granulomatous disease. It has a mortality of up to 5%. and are responsible for the destruction of antibo dycoated target cells. pH measured in pH probe E. Carbon monoxide . pH increases because of excretion of bicarbonate 108-A 16 year old girl is admitted with progressive weight loss for which no organic cause can be found. superoxide anion and nitric oxide within activated macrophag es and neutrophils upon phagocytosis. NK cell killing 4.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 107 Theme: Acid/base balance A. this should be excluded when making a diagnosis of anorexia. Normal pH absolutely essential for enzyme function/ion distribution/protein stability D.FasL Fas-FasL interaction leads to apoptosis of the Fas expressing cells. A low plasma potassium may result from laxative use or self-induced vomitting.hyperbaric oxygen 2.they use this question a lot C. In pregnancy. fine lanugo hair. This process is catalysed by an enzyme complex NADPH oxidase and is important in killing the engulfed microorganisms. A history of laxative abuse 2. Intracellular pH = extracellular pH . pH 7. 110-The following poisons are matched to the appropriate therapy: 1. An excess of physical exercise 4. a process termed antibody -dependent cell-mediated cytotoxicity (ADCC). CTL-mediated lysis 2. CD16) which bind to the constant region of IgG. The oxidative burst refers to the process of generation of toxic products such as hydrogen peroxide. Defects in the NAPDH oxidase pathway leads to susceptibility to bacterial and fungal infections.atropine and pralidoxime 84 . Natural killer cell s destroy cells by the release of cytoplasmic granules containing perforin and granzymes. Self-induced vomiting 5. Oxidative burst within activated macrophages 5. A delusion of outside control implies psychosis.35-7. Low plasma potassium Teaching Notes for Question 108 Theme: Anorexia Anorexia Nervosa most commonly occurs in young women. The delusion that someone is trying to control her eating patterns 3. They have abundant Fc gamma receptors on their surface (FcgammaRIII.

thyroid peroxidase catalyses the 2nd and 3rd steps. calcium EDTA ± dimercaprol for lead poisoning. Methanol .ethanol 5. sequences not found in the bacterium's own DNA). Coupling of 2 DIT residues gives T4 while addition of MIT and DIT gives T3. and temporary pacing. and a beta -blocker for treatment of SVTs. try atropine. Are useful in linked-gene diseases 4. dicobalt edetate for cyanide. *TSH stimulates all steps in thyroid hormone synthesis . 3. About 99% of circulating T4 is protein bound 4.PEDIATRICS EXAMINATION REVIEW 3. Other antidotes include desferrioxamine for iron. the enzyme will not cut and 85 . ethanol for ethylene glycol. These enzymes are produces by bacteris (probably as a defence against viruses) and cut DNA at specific sequences. Are found every 100 bases 2. naloxone for opiates. Are detected by Northern blotting Teaching Notes for Question 112 Theme: Restriction fragment length polymorphisms The term restriction fragment length polymorphisms (RFLP) refers to the different patterns seen when DNA from different persons is digested by bacterial enzymes (restriction enzymes). *Both T4 and T3 are highly bound to thyroid binding globulin or albumin. which is concentrated to 20 times that of plasma. Active uptake of iodide. 112-Restriction fragment length polymorphisms (RFLPs): 1.phentolamine 4. N -acetylcysteine for paracetamol. glucagon infusion. T3 is roughly 10 times more potent than T4 5. and vitamin K for warfarin. The uptake of iodide into the thyroid follicular cell is by active transport 2.phenytoin Teaching Notes for Question 110 Theme: Antidotes For beta-blocker overdose. Tricyclic antidepressant overdose may require intravenous neostigmine to counteract the anticholinergic effects. All steps in thyroid hormone synthesis are stimulated by thyroid peroxidase Teaching Notes for Question 111 Theme: Thyroid hormones The main steps are as follows: 1. 111-The following are true: 1. Are generated by digestion of DNA with bacterial enzymes 5. Are useful in diagnosis of multifactorial diseases 3. *T4 has a half-life of 7 days T3 is 1 day. Fuller's earth for paraquat. Tricyclic antidepressants . Beta-blocker . the DNA is cut only where the particular sequence occurs producing fragments of DNA. 2. T4 has a half-life of 24 hours 3. Oxidation of iodide to iodine. and then the patterns of different fragment sizes compared from on e sample to another. W hen human DNA is subjected to digestion. If a mutation changes the DNA sequence. which combines with ty rosine residues to give monoiodotyrosine (MIT) and di-iodotyrosine (DIT). These can be resolved according to size by gel electrophoresis. *T3 is 3 to 5 times more potent than T4. dimercaprol for heavy metal poisoning. digoxin specific antibody for digoxin.

g.increasing macrophage antimicrobial activity. It is bicuspid 86 . Northern blotting is used for the analysis of RNA not DNA. These cells tend to be CD4 and CD8 negative. Lymphadenopathy is common because of the over -expansion of B-lymphocytes Teaching Notes for Question 114 Theme: In the athymic child The thymus is the major organ of development of both helper and cytotoxic T -cells. * Quantitative – which is data to which a certain number can be ascribed. T-cells play a vital role in macrophage function. number of children or cont inuous (assuming any value) e. the thymus fails to develop and is associated with par athyroid agenesis (hence tetany) and congenital defects of the heart and great vessels. 113-Which of the following statements are true: 1. Discrete data may include number of children 3. there is some evidence that they are able to develop in athymic mice possibly in the intestinal mucosa. This can be either discrete (only discrete values are assumed) e.g. TNF-beta) and by secreting cytokines e. Humoral responses are integrally dependent on T -cell help.g. IFN-gamma . Macrophage function is impaired 2. Patients with residual thymic tissue may have some T-cell function which tends to improve with age. The development of gamma/ delta -T-lymphocytes is unaffected 3. and therefore antibody production and B -cell function are affected when the thymus is absent. This can be either nominal (a naming scale) e. Nominal data may include blood group type Teaching Notes for Question 113 Theme: Types of data Data may be described as: * Qualitative – only a quality can be applied and not an exact number. In humans <5%of T-cells express the gamma/delta T-cell receptor. Data may be described as qualitative or quantitative 2.g. 115-The following statements are true about the mitral valve 1. 10% of intestinal T-cells express the gamma/ delta receptor. The absence of thymus affects the development of both alpha/beta and gamma/delta T -cells. many have no T-cell defects by adolescence. The function of helper T-lymphocytes (Th) is affected while cytotoxic T -lymphocytes (Tc) activity is only moderately impaired 5. Although these cells a re thymus dependent. T-helper cells activate macrophages by directly interacting with these cells via costimulatory molecules (CD40L. In humans. blood group type or ordinal (data can be ranked in some way) e. Gamma/ delta T-cells in mice are found predominantly in epithelial tissues. TNF -alpha. Discrete data may include serum glucose values 4. Ordinal data does not include tumour staging 5. but further detailed analysis of the exact m utation will still be necessary to identify the gene responsible. The production of antibody is unaffected 4. plasma glucose. In DiGeorge. tumour staging. RFLPs can be used to link phenotype (disease) to genotype (sequence). Much of what is known about gamma/delta T -cells is derived from studies in mouse.g.PEDIATRICS EXAMINATION REVIEW so the sizes of the DNA fragments will be different. This is due to the failure of development of the 3rd and 4th pharyngeal pouches. 114-In an athymic child:1.

Rubella . SLE 4. scarlet fever. poxviral infections and Impetigo. Roseola Infantum 2. Hand Foot and Mouth disease 4. Rheumatoid arthritis and thyrotoxicosis are all causes of lymphadenopathy. SLE. Dermatitis herpetiformis 3. Kawasaki disease 5. Thyrotoxicosis Teaching Notes for Question 117 Theme: Lymphadenopathy Rubella. It can be regurgitant even if structurally normal Teaching Notes for Question 115 Theme: Mitral valve anatomy and physiology The mitral Valve has two cusps and large papillary muscles to hold the cusps firmly. Measles. Opening is only audible in mitral stenosis (as the opening snap). Angiotensin II promotes renal arteriolar dilatation 3. 116-Causes of vesicular rash in children include:1. Closure of the mitral valve is associated with the first heart sound. Herpes simplex type I. Rubella 2. 118-The following statements are true for the Renin Angiotensin System: 1. The associated papillary muscles are smaller than those of the tricuspid valve 4. Angiotensin II inhibits aldosterone secretion by the adrenal glands 87 . Its' opening is associated with the first heart sound 5. Sarcoidosis but not Amyloidosis is also a cause of lymphadenopathy. These muscles are larger than those for the tricus pid valve as the LV pressure is higher. Kawasaki disease. Amyloidosis 3. Rheumatoid arthritis 5. Renin converts Angiotensin I to Angiotensin II in the kidney 2. roseola infantum and other viral infections present with maculopapular rash 117-The following are causes of lymphadenopathy: 1. Molluscum contagiosum Teaching Notes for Question 116 Theme: Vesicular Rash Other causes of vesicular rash in children include Varicella and Herpes zoster. It lies posterior to the sternum at the level of the fourth costal cartilage 3.PEDIATRICS EXAMINATION REVIEW 2. even wit h a structurally normal valve. Mitral regurgitation can occur with dilatation of the left ventricle. erythema infectiosum.

* Guillain-Barré syndrome. tetracycline. * Head trauma. 120-The following is true regarding endotoxin: 1. esp of the efferent arteriole. * Sinusitis. * Polycythemia. · Angiotensin II promotes renal arteriolar constriction. In spite of all these. Angiotensin II receptors are blocked by spironolactone 5. If the pressure is high. It is idiopathic i n 50%. · Aldosterone receptors are blocked by Spironolactone · Captopril inhibits ACE. Papilloedema is a common early finding. vitamin A and oral contraceptives). others include diplopia. if a child is still symptomatic with progressive loss of vision. * Hypoparathyroidism. It is heat-stable 3. Seizure Teaching Notes for Question 119 Theme: Benign intracranial hypertension Benign intracranial hypertension is characterised by increased intracranial pressure in the absence of a mass lesion or hydrocephalus. It is a protein 2. some may have nausea. * SLE. Lumbar puncture will help to relieve headache and preserve the vision. Less commonly. Losartan inhibits formation of Angiotensin II Teaching Notes for Question 118 Theme: Rennin Angiotensin System · Renin converts angiotensinogen (produced in the liver) to Angiotensin I. the papilloedema will progress and cause optic nerve atrophy. paraesthaesia. 119-Which of the following are common sympt oms associated with raised intracranial pressure in children? 1. * Adrenal insufficiency.the normal pressure lies between 12-16 mmHg. neck stiffness. Abdominal pain 3. * Hyperadrenalism. Losartan is a competitive antagonist of the A II receptor. · Angiotensin II also stimulates aldosterone secretion leading to Na and water absorption. Morning headache 2. * Hyperthyroidism. Cranial CT or MRI is essential with ophthalmology review.PEDIATRICS EXAMINATION REVIEW 4. caused by 6th nerve palsy (abducens). * Iron deficiency anaemia. * Otitis media. Ataxia 5. it should be reduced by 40% . Acetazolamide can be started if patient cannot tolerate second or third lumbar puncture when symptoms recur. dexamethasone can be given for two weeks or/and consider optic nerve fenestration or lumboperitoneal shunt. It is produced by some Gram -positive bacteria 4. Secondary causes include: * Drugs (corticosteroids. If not treated. All endotoxin has the same physiological effect 88 . Double vision 4. and ataxia. vomiting. Headache is the commonest feature. which is converted to Angiotensin II by the Angiotensin Converting enzyme (ACE) in the lungs. * Leukaemia.

Infection in pregnancy can result in fetal anemia 5. sheep (Echinococcus granulosus) Leptospirosis (Weil’s) Lyme disease Rabies Toxocariasis Toxoplasmosis Rats Deer. Poliomyelitis is found only in humans. cooling towers. Aplastic crises due to parvovirus infection in sickle cell disease usually requires bone marrow transplant Teaching Notes for Question 122 Theme: Parvoviris B19 Parvovirus B19 infection may be asymptomatic or cause a condition called erythema infectiosum(Fifth disease/Slapped cheek disease). bats Dogs. cattle. sheep. etc. puppies. Organisms may be bacteria. It is secreted by bacteria during active infection Teaching Notes for Question 120 Theme: Endotoxin Endotoxin is composed of lipopolysaccharide in the Gram -negative cell wall. cattle. cattle. 122-Regarding Parvovirus infections in children: 1. pigs Sheep. protozoa. Chronic anemia can be a manifestation in some children 4. kittens Salmonellosis Q fever (Coxiella) Poultry. the effects of endotoxin are all non -specific. Hydatid disease may present to a surgeon as liver cysts. cats Cats Legionnaires’ disease (organism Legionella pneumophila) exists in water systems. jacuzzis etc. Important zoonoses include: Disease Animal source Campylobacter Cattle. Hydatid disease 3. foxes. poultry. sheep Cryptosporidiosis Sheep. chlamydiae. Legionnaires’ disease 2. It is the causative agent for exanthem subitum 2. Lyme disease 4.zoonotic infections. Whereas exotoxin is secreted by viable bacteria.PEDIATRICS EXAMINATION REVIEW 5. puppies. wild rodents (via tick bites) Dogs. endotoxin is released only following bacterial cell lysis or death. 121-The following are zoonoses: 1.It may present with fever/ malaise/rash 89 . Poliomyelitis 5. goats Psittacosis Birds Hydatid disease Dogs. Arthritis is a very common manifestation in children 3. Salmonellosis Teaching Notes for Question 121 Theme: Microbiology . A zoonosis is an infection that is naturally transmissible between vert ebrate animals and man. viruses. Unlike exotoxin.

2. Usually blood transfusions are all that is needed. A child should kick as well as jump at 2 years. 5. Raised left atrial mean pressure. Not run until 2 years Teaching Notes for Questi on 124 Theme: Milestones Importantly. Rapidly repeating tonic -clonic seizure with not returning to full consiousness Teaching Notes for Question 123 Theme: Epilepsy * Status epilepticus is d efined as a condition in which continuous or rapidly repeating tonic-clonic seizures persist for 30 minutes or more. Cruise by 1 year 5. It specifically affects erythrocyte precursors and in conditions with shortened life span like Sickle cell disease/Thallasemia/Hereditary spherocytosis and other hemolytic anemias it can cause aplastic crises which resolves spontaneously in 2 to 3 weeks. Roll over by 2 months 2. * It is often associated with incontinence but this is required for the diagnosis. Tonic-clonic seizure lasting 30 minutes with incontinence 5. 3. 4.PEDIATRICS EXAMINATION REVIEW over face followed by rash over trunk. Ride a tricycle at 2 years 3. Hypercapnia. Reduced pulmonary vascular resistance. a normal child would be expected to: 1. a child won’t roll at 2 months as spuriously suggested by perpetrators in cases of non accidental injuries. Unresponsive patients who have been incontinent for 60 minutes 3. In pregnancy it can cause fetal hydrops and fetal anemia. Running is usually achieved by 18 months. Reduced lung compliance. Mouth twitching lasting 60 minutes with reduced GCS 4. mouth twitching is a common sign of late status. 123-Definition of status epilepticus: 1. 125-The functional consequences of moderately severe mitral s tenosis include: 1. This milestone is at around 4 months. Two or more fits within 30 minutes 2. * As the seizures persist they often become localized. 90 . Reduced glomerular filtration rate. Cruising is moving around the round by holding on to furniture before in dependent walking begins and can be expected by 1 year. In children with leukemia it can cause prolonged anemia. Can cause transient arthritis. Riding a tricycle is expected at the 3 year . Kick a ball at 2 years 4. but there are many other diagnosis in a patient with mouth twitching and reduced GCS 124-Regarding Gross Motor Development.

protein content of 500mg/day is within normal range 5. normal pH is >5.5mg/kg/hr 128-In children with renal failure 91 . IL-2 : activation induced cell death 3.it inhibits macrophage production of IL -12. It is an inhibitory cytokine . IL-6: induction of antibody secretion by plasma cells 5. 127-Urine 1. normal osmolality is 300 -600 mosmol/kg 4. It stimulates the growth and cytolytic activity of NK cells and high concentrations can produce lymphokine -activated killer cells (LAK cells).5 2. It promotes differentiation of B -cells into plasma cells and increases antibody production. Reduced cardiac output reduces renal perfusion thereby reducing glomerular filtration rate. IL10 is produced by macrophages and some Th2 cells. vascular endothelium and bone marrow stromal cells. IL-12 is produced by macrophages and dendritic cells. IL-10: activation of B-cells 4. IL-6 is produced by macrophages. TNF and other cytokines. functional minimum urine output/day in 70kg male is 1400mls Teaching Notes for Question 127 Theme: Urine biochemistry 1 normal <5. IL-12: induction of IFN-gamma secretion by activated T-cells Teaching Notes for Question 126 Theme: Cytokines Cytokines often have many functions. and may act as a co-stimulatory signal for naive CD8+ T. Paradoxically IL -2 is also important in activation induced cell death (AICD). It is a potent inducer of IFN -gamma production by T-cells and NK cells. It stimulates the growth and activation of T -cells. T-cells. a form of apoptosis. It promotes the differentiation of Th0 cells into Th1 cells and is the most important cytokine implicated in this lineage decision. IL-2 is produced by T-cells.cells. depending on the range of target cells: IL-3 is produced by T-cells.one of the most basic renal functions is Na conservation 3 4 150mg / day is normal maximum 5 500mls is obligatory minimum and oliguria defined as <0. Pulmonary vascular resistance is increased due to raised left atrial pressures and chronic hypoxiemia 126-Evaluate the truth of the following descri ptions of cytokines and their functional activities:1. It is a growth factor for progenitor haematopoietic cells (all types). NK cells and mast cells. normal Na content is <20mmol/L 3. fibroblasts. It also reduces expression of costimulatory molecules and MHC class II molecules on these cells.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 125 Theme: Effects of mitral stenosis on heart physiology Chronic pulmonary venous congestion leads to reduced lung compliance thereby increasing the work of breathing. IL-3 : growth and differentiation of bone-marrow stem cells 2.3 2 true . It also induces synthesis of acute phase proteins by hepatocytes and promotes myeloid stem cell differentiation.

protein restriction is usually inappropriate 129-Lung surfactant 1. Lymphopenia is common. RSV. Ribavarin is curative. Appears only after the 1st week of life Teaching Notes for Question 129 Theme: Surfactant Surfactant is a dipalmitoyl-phosphatidyl choline and is a phospholipid. Is a glycoprotein 4. 4. It is produced by type -II pneumocytes and is seen at about 24 weeks gestation. In children with CRF since growth failure may occu r and the metabolic demand is high. which prevents alveolar collapse by reducing alveoli surface tension. Causes an increase in chest wall compliance 3.IG is recommended for infants less than 2 yr with chronic lung disease or prematurity but shou ld not be given to those with CCHD because of increased complications. RSV immune globulin is recommended in infants with cyanotic congenital heart disease to prevent severe RSV infection. 130-With regards to bronchiolitis.CXR 92 . It is given IV/ IM just prior to and during RSV season. 3. Ribavarin. thrombocytaemia. 2. and uraemia. drug OD. hypertension. acidosis. Maintains the same surface tension for diffe rent sized alveoli 5. Decreases the surface tension within an alveolus 2. But there is no convincing evidence to suppo rt its use. It is linked to maternal smoking. CXR commonly shows hyperinflation. microangiopathic haemolytic anaemia is characteristic 5. It causes an incre ase in lung compliance only (not chest wall compliance). Teaching Notes for Question 130 Theme: Bronchiolitis Infants whose mothers smoke are more likely to get bronchiolitis than those of non smoking mothers. The WBC count and differentials are usually within normal limits in bronchiolitis. Protein intake should be restricted in chronic renal failure. the following are true : 1. 5. In acute renal failure. dialysis is indicated with rapidly rising sodium 2. an antiviral drug may be given to infants with cong heart disease or BPD. 40% resolve spontaneously. Anaemia of chronic renal failure is due to low plasma ferritin levels 3. involving anaemia. This helps to minimise uraemia Usually seen post diarrhoea (especially E Coli 0157). Grade V vesico-ureteric reflux spontaneously resolves in only 10% Teaching Notes for Question 128 Theme: Renal failure Rising K+. In haemolytic uraemic syndrome. and rising Cr are all indications (if they are unresponsive to treatment) Erythropoitin is made in the kidneys. 4. which stimulate s red cell production. grades I -III 85%.PEDIATRICS EXAMINATION REVIEW 1.

35 .45 = [H+] 36 – 45 nmol/L. In pregnancy. pH 7.7. Not essential to maintain ion distribution. 5. however the followi ng list details the important functions: Cholecystokinin (duodenum & jejunum) increases gall bladder contraction Increases colonic motility Gastrin (antrum & duodenum) increases gastric acid secretion Increases GI mucosal growth Secretin (Duodenum & Jejunum) increases pancreatic bicarbonate production VIP (enteric nerves) increased intestinal secretion Splanchnic vasodilation Pancreatic polypeptide (pancreas) reduces biliary and pancreatic secretion Peptide YY (ileum & colon) inhibits pancreatic ex ocrine function Neuropeptide Y (enteric nerves) regulated intestinal blood flow Motilin (whole gut) increases small bowel motility Bombesin (gut & Pancreas) stimulated pancreatic exocrine activity Somatostatin (stomach & pancreas) inhibits secretion an d action of gut hormones Glucagon (pancreas) gluconeogenesis and reduces GI motiltiy 132-Normal arterial pH is: 1. Normal pH absolutely essential for enzyme function/ion distribution/protein stability.46 nmoles/L. Scattered areas of atelectasis may be seen in approx 30%. Pancreatic polypeptide inhibits pancreatic secretion Teaching Notes for Question 131 Theme: GI hormones The roles of individual gut hormones are not completely defined. Equal to [H+] of 34 . Lower than intracellular pH. Glucagons increases GI motility 4. Decreased in pregnancy. 2. 4. 131-The following concerning gastrointestinal hormones are true 1. Somatostatin reduces gastric motility 2.PEDIATRICS EXAMINATION REVIEW commonly reveals hyperinflation. pH increases if anything because of excretion of bicarbonate. 3. Haemophilus influenzae 93 . Epstein Barr virus 2. Adenovirus 4. Teaching Notes for Question 132 Theme: Acid/base balance 1. 133-The following organisms cause conjunctivitis: 1. Gastric distension increased motility 3. 4. 2. pH measured in pH probe. Intracellular pH = extracellular pH . 3.they use this question a lot. Measured rather than calculated in a pH probe. Chlamydia trachomatis 3. Secretin inhibits pancreatic bicarbonate secretion 5. 5.

Anaemic patients should receive regular iron supplementation 5. There are 18 bronchopulmonary segments 3. Don’t forget sexual health screening for the mother and informing public health of ophthalmia neonatorum. In the homozygous state. The right middle lobe extends from the 5th rib in the axilla to the 4th costochondral junction superiorly and to the 6th costochondral junction inferiorly 135-In beta thalassaemia:1. is diagnosed on gram stain and culture and should be treated with IV penicillin and chloramphenicol eye drops. endocrine dysfunction. The vagus nerve is responsible f or normal bronchial tone 2. Adenovirus causes conjunctivitis in summer outbreaks. 4. There are 5 lobes divided into 18 segments. and iron overload resulting from transfusion and increased iron absorption. Neisseria gonorrhoeae Teaching Notes for Question 133 Theme: Organisms causing conjunctivitis Chlamydia trachomatis causes conjunctivitis in 30 -50% of neonates born to mothers with cervicitis. which develops 5 -14 days after birth and is indistinguishable from gonococcal infection. Diagnosis is by haemoglobin electrophoresis. The anaemia becomes manifest in late infancy. At birth there are 25 million alveoli. Most of the alveoli in the adult lung are present at birth 4. Tetracycline ointment topically is combined with oral erythromycin – the oral antibiotic is to prevent relapse after ointment is discontinued and to prevent progression to pneumonia.PEDIATRICS EXAMINATION REVIEW 5. beta thalassemia (ie. Diagnosis can be made by Haemoglobin electrophoresis 4. thalassemia major) causes severe transfusion -dependent anemia with high reticulocyte count. The reticulocyte count is low 3. Gonococcal conjunctivitis presents earlier than chlamydial disease (usually within 2 days). The major component of surfactant is dipalmitoyl lecithin Teaching Notes for Question 134 Theme: Anatomy 2. It is diagnosed on a swab scraped over the lower eyelid (to allow cells to be collected – don’t forget it is an intracellular organism) by direct fluorescent antibody. 134-The following statements are true: 1. 94 . Beta thalassaemia major presents at birth with profound anaemia 2. enterovirus. ELISA or PCR. 3. which increase in the adult to 300 million per lung. ineffective erythropoiesis. liver dysfunction. extramedullary hematopoiesis. coxsackie and herpes simplex are other viral causes. The surface markings of the right middle lobe approximate to the axilla 5. cardiac dysfunction) o The physical findings are related to severe anemia. This overload causes clinical problems similar to those observed with primary hemachromatosis (eg. Increased iron deposition resulting from multiple life -long transfusions and enhanced iron absorption results in secondary iron overload. It is a purulent conjunctivitis. Splenectomy is contraindicated due to extramedullary haemopoiesis Teaching Notes for Question 135 Theme: beta thalasseamia Beta thalassemia syndromes are a group of hereditary disorders characterized by a genetic deficiency in the synthesis of beta -globin chains.

Hepatomegaly related to significant extramedullary hematopoiesis typically is observed. 2. the commonest being astrocytomas. breast carcinoma and malignant melanoma. In asymptomatic children with increased lead levels DMSA therapy is recomme nded 3. usually multiple.PEDIATRICS EXAMINATION REVIEW o Skin may show pallor from anemia and jaundice from hyperbilirubinemia. 4. o The skull and other bones may be deformed secondary to erythroid hyperplasia with intramedullary expansion and cortical bone thinning.20% are meningiomas. Teaching Notes for Question 136 Theme: Central nervous system Around 80% of brain tumours a re primary. Astrocytomas arise from the neuroglia. Urinary lead concentration is the most useful indicator of exposure 2. 136-The following statements concernin g CNS tumours are true: 1. o Heart examination may reveal findings of cardiac failure and arrhythmia. 3. are bronchial carcinoma. transfusion -associated viral hepatitis resulting in cirrhosis or portal hypertension also may be seen. Splenomegaly typically is observed as part of the extramedullary hematopoiesis or as a hypert rophic response related to the extravascular hemolysis.50%) arise from the glial cells. which metastasise to the brain. Around 15 . Pituitary tumours are around 5% of the total. usually benign and a rising from the meninges. o Extremities may demonstrate skin ulceration. and thyroid. The gall bladder may contain bilirubin stones formed as a result of the patient's life-long hemolytic state. Meningioma is usually benign. Gastric carcinoma frequently metastasises to the brain. 75% of lead in children is in the bone. gall bladder. Chelation removes lead from the soft tissue pool. related to either severe anemia or iron overload. and spleen. The half-life of the deep bone compartmen t is months 5. 15 . Cerebral metastases comprise around 70% of all brain tumours. 5. Patients who have received blood transfusions may have hepatomegaly or chronic hepatitis due to iron overload. and soft tissue pool. Lead poisoning is associated with basophilic stippling of red blo od cells. Haemodialysis is useless at eliminating lead because of the large non -blood pools.glioblastoma multiforme is the most malignant subtype. The deep bone pool has a half life of decades. testes. 95 . Glioblastoma multiforme is a benign neoplasm. Asymptomatic children with lead levels >46µg/dL are treated with courses of DMSA (dimercaptosuccini c acid) for 2 weeks followed by a 2 week interval to assess post-treatment rebound which occurs due to equilibration from bone stores. especially affecting the pancreas. 137-Regarding lead poisoning:1. Haemodialyisis effectively eliminates lead 4. These are of variable malignancy . o Abdominal examination may reveal changes in the liver. The most frequent tumours. Is associated with basophilic stippling in white cells Teaching Notes for Questio n 137 Theme: Lead poisoning Unlike almost all other trace metals blood rather than urine concentrations are the most useful for assessment of lead exposure.25% are brain secondaries. There are 2 bone pools a shallow and a deep in addition to the blood. The most frequent (around 40 . o Iron overload also may cause endocrine dysfunction.

Has a poor prognosis 3. Is a common form of partial epilepsy 2. Mobilisation of B-cells from the bone marrow 3. activated T-cells. 3. 15-20% of childhood epilepsy. 139-Which of the following biological responses or consequences is mediated by the release of tumour necrosis factor (TNF) –alpha? 1. TNF acts as an endogenous pyrogen by increasing energy mobilisation in fat and muscles. 2. It activates phagocytes. arms. Disseminated intravascular coagulation in systemic Gram -negative bacterial infection 4. TNF causes circulatory collapse associated with vasodilatation. may not require treatment.g.PEDIATRICS EXAMINATION REVIEW 138-Benign rolandic epilepsy :1. and chronic production suppresses bone marrow haematopoiesis. Treatment with carbamazepine. At higher concentrations. activated NK-cells and occassionally mast cells. Production of acute phase proteins by hepatocytes 2. Seizures usually occur in sleep: sensory aura followed by GTC seizures often involving face. Teaching Notes for Question 140 Theme: Head-Neck 96 .the most common parti al epilepsy in childhood. Stellate ganglion. Phrenic nerve. Scalenus anterior. 5. It has a wide range of systemic effects. It also produces cachexia by inducing appetite suppression. Induction of killing by cytotoxic T lymphocytes (CTL) Teaching Notes for Question139 Theme: Tumour necrosis factor (TNF) TNF-alpha is produced by mononuclear p hagocytes. Can be successfully treated with carbamazepine 5. Dendritic cell migrati on and maturation is enhanced. It is a procoagulant. The superior intercostal artery. decreased myocardial contractility a nd intravascular thrombosis. 140-The neck of the 1st rib is related to: 1. in gram-negative bacterial sepsis. enhancing cytokine production and microbicidal activity. At extremely high concentrations e. Has a typical EEG with a 3 per second spike and wave pattern Teaching Notes for Question 138 Theme: Epilepsy Benign rolandic epilepsy. Seizures usually stop in mid teens. Excellent prognosis. TNF increases the expression of adhesion molecules on vascular endothelium and results in increased microvascular endothelial permeability to facilitate recruitment of inflammatory cells It increases neutrophil adhesion molecule expression and mobilises neutrophils from the bone marrow. Involves seizures that often occur at school 4. Resetting of the temperature threshold in the hypothalamus resulting in pyrexia 5. and increases synthesis of acute phase proteins. The thoracic duct on the right. 4. EEG shows typical cento -temporal spikes.

Structures crossing the neck of the first rib from me dial to lateral are: 1. which makes it different from peripheral neuropathy which is not dermatomal. temperature and preservation of light touch 4. Is sometimes related to poor parenting 3. It is resected. The first rib is flattened from above downwards. Is related to Fragile X syndrome 4. Can be treated successfully with psychotherapy 2. Loss of pain. 142-A patient develops low back pain which is progressive. 143-Autism :1. Loss of tendon reflex 3. Alveolar haemorrhage. Extra-pulmonary restriction 2. A BRANCH OF T1 THAT CONTRIBUTES TO THE BRACHIAL PLEXUS. A prominent tubercle on the inner border of its upper surface marks the insertion of scalenus anterior. Churg-Strauss 3. Loss of motor function 2. Posterior division causes spinothalamic tract degeneration. The thoracic duct is only present on the left side. as this is provided by the ganglia. but division of the posterior spinal root occurs. Spinothalamic tract degeneration abo ve the section level 5. Exercise 5. THE SYMPATHETIC TRUNK 2. Which of the following is likely to result from this? 1. He is found to a spinal schwannoma. ( and ASTHMA). and usually not entirely sensory or motor. Goodpasture’s syndrome 4. There is no effect on the sympathetic supply. It is the shortest flattest and most curved of the ribs. Exercise increases pulm onary blood flow. Anaemia Teaching Notes for Question 14 1 Theme: Increase in CO transfer factor Any condition that increases the number of red cells that come into contact with the carbon monoxide will increase the TLCO and KCO. Alveolar inflammation.PEDIATRICS EXAMINATION REVIEW Stellate ganglion is formed by the c8 -t1 nerves. Extra-pulmonary restriction would increase the density of blood per unit lung volume with more red cells coming into contact with the CO. Absence of sweating in the limb Teaching Notes for Question 142 Theme: spinal nerve root lesion Division of dorsal/posterior root usually causes loss of all sensory modalities in a dermatomal distribution. 141-Which of the following would cause an increase in the carbon monoxide transfer factor? 1. THE SUPERIOR INTERCOSTAL ARTERY 3. Is related to Sandifer Syndrome 97 .

144-With regards the spleen: 1.either Penicillin V bd or Amoxycillin od. use of gestures. Initially normal development with regression and loss of skills before 2 years of age. Autistic features. Following splenectomy. 4. immunological theories. Imagination: restricted range of interests. These are then presented to B-cells and an appropriate immune response is initiated. 5. Adult doses: Penicillin V 250 mg bd. All patients regardless of underlying condition. Poor symbolic or imaginative play. I nability to recognise or appreciate the feelings of others and respond to social cues. Parents often report child not wanting to be cuddled as an infant.PEDIATRICS EXAMINATION REVIEW 5. Patients with asplenia or hyposplenia (sickle cell disease. The risk does not diminish with time post -splenectomy. Inflexible routines. Asperger’s syndrome: actually modern view suggests that Asperger’s describes the milder autistic children ie an autistic spectrum of disorders exists. it is largely the kupffer cells in the liver that filter micro -organisms.echolalia. rocking. ritualistic behaviour. Following splenectomy. Co-Trimoxazole may be an alternative for children. Causes: genetic (rate of autism in siblings increased. An area called the marginal zone is closely associated with this periarteriolar lymphoid sheath (PALS) and is rich in B -cell follicles. should be on life -long antibiotic prophylaxis . Poor development of facial expression. The spleen is involved in the destruction of encapsulated organisms. infective aetiology. Macrophages located in the marginal zones of the spleen are very efficient at trapping and retaining polysaccharide antigens. which contains an abundance of erythrocytes. Late development of social smile. Poor speech development. and death may occur in hours. Teaching Notes for Question 144 Theme: Spleen The spleen is specialized to trap and mount an immune response against blood borne antigens. Unable to modify their behaviour on the basis of these social cues. hand flapping. abnormal use of speech . Inability to adapt to change. child abuse or neglect. Treatment of developmental disorders: symptomatic treatment as causes poorly defined. low dose penicillin & pneumovax are recommended. CLL) or who have concomitant liver disease. but is not recommended in adults. 98 . Stereotypies eg head banging. Is related to Rett syndrome Teaching Notes for Question 143 Theme: Autism and pervasive developmental disorders Features impairments in socialization. with a prefe rence for Pencillin V. autism is a feature of f ragile X). Children's dosage is 10mg/kg. communication and imagination. Amoxycillin 500 mg od. It is comprised of the red pulp. Communication: verbal and non -verbal language affected. 2. It is highest in those who h ave had a splenectomy for lymphoproliferative disease (NHL. The onset of symptoms may be extremely rapid. Basophilic stippling of neutrophils is seen with asplenia. More common in boys than girls. Poor development of joint attention. The marginal zone and PALS are populated by interdigitating dendritic cells specialized in the presentation of antigen to T -cells. The lymphoid function is carried out in the white pulp. Rett’s syndrome: occurs in girls. protodeclarative pointing. some patients with coeliac disease) are at increased risk of overwhelming bacterial infection. Gaze avoidance. Usually diagnosed around 2-3 years of age as that is when speech delay becomes readily apparent. The white pulp forms a sleeve around penetrating arterioles and contains numerous T cells. especially those with congenital asplenia. Educational and behavioural methods are mainstay of treatment. NOT related to parenting methods. especially language and manual dexterity skills. Social interaction: ‘autistic aloneness’. 3. They tend to be diagnosed later because they a re able to cope and adapt better than severe autistics. Asperger’s describes those children who have impairments in the core features without learning difficulties.

Patients are susceptible to infection with bacteria and viruses 99 . erythromycin should be used (250 mg bd adult dose – 10 mg/kg child). The eosinophils to the lung are recruited from the bone marrow 2. spleen. Basophillic stippling is seen in lead poisoning. It is often a slowly progressive disease and should not be treated in the early stages. Howell Joly bodies are seen in asplenia. but one third of patients die of causes other than their CLL. 147-With regards severe combined immunodeficiency: 1. IL-5 is produced by T helper cells (TH-2) and recruits eosinophils from the marow. All other patients should receive the 3 vaccines even after splenectomy. 5. All adult patients should receive an annual influenza immunization. 146-In asthma:1. Teaching Notes for Question 146 Theme: Asthma. Should be treated aggressively in the early stages of the disease 4.protein conjugate (either tetanus or diphtheria conjugates) and meningococcal A & C polysaccharide vaccines at least one week before surgery. Patients have defective T-cell dependent antibody responses and defective cell -mediated immunity 2. Has a median survival of less than 5 years 5. after a blood culture (if possible). Is asymptomatic at diagnosis in less than 10% of patients 3. 145-Chronic lymphocytic leukaemia :1. haemophilus influenzae type B capsular polysaccharide . Annual hospital follow-up is recommended. Is associated with smear or smudge cells in the peripheral blood 2. Intravenous penic illin. Median survival is 3-5 years. Bronchial hyper-responsiveness (BHR) does not have to be present. Any asplenic/hyposplenic patient who develops a sudden febrile illness should be treated promptly with full dose antibiotics. Patients travellin g to malarial areas and areas where penicillinresistant pneumococci have been found require specialist advice. Steroids are contra-indicated if the patient is a diabetic 3. Booster immunization may be required if levels of antibody fall below protective levels. 3 weeks after and then at yearly intervals thereafter. Patients undergoing elective splenectomy should receive pneumovax. pathophysiology In asthma. Blocking interleukin-5 maybe therapeutic 4. It is asymptomatic at diagnosis in over 30% of patients. Smear or smudge cells are often seen in the peripheral blood which represent the fragile malig nant cells which have been squashed as the blood film is spread. Leukotriene inhibitors are associated with the development of Churg -Strauss . and splenomegaly is seen in 50% at diagnosis. Is associated with splenomegaly at diagnosis in about 50% of patients Teaching Notes for Question 145 Theme: Chronic lymphatic leukaemia Chronic lymphatic leukaemia (CLL) is due to an accumulation of mature lymphocytes in the peripheral blood.PEDIATRICS EXAMINATION REVIEW For Penicillin-allergic patients. liver and lymph nodes. Blocking IL -5 will therefore be therapeutic. by definition bronchial hyper -responsiveness must be present. as there may be some benefit. Leukotriene inhibitors are associated with Churg -Strauss syndrome and causality can not be excluded. bone marrow. should be started immediately. Blood levels of antibodies to these polysaccharides s hould be measured before.

Should recognise his/her name 2. 7. Progesterone 3. purine nucleoside phosphorylase (PNP) deficiency. T-B+ SCIDs may also be due to an autosomal recessive mutation in Jak 3 –an intracellular enzyme which is a component of the signalling pathway induced by various cytokines. 149-A normally developing 18 -month-old child: 1. Plays in parallel with his/her peers 4. all of which are autosomal recessive. Ganglion blockers 5. 148-Lower esophageal sphincter tone is decreased by: 1. Other known causes of T -B. Cisapride 2. Ondansetron Teaching Notes for Question 148 Theme: Esophageal sphincters The effect of progesterone on oesophageal sphinter tone is one of the many reasons for the increase in gastro oesophageal reflux in pregnancy. X-linked SCID is caused by a defect in the IL -2 receptor 4. Suxamethonium 4. Should be able to copy a vertical line Teaching Notes for Question 149 Theme: Developmental milestones 100 .and B-cells.and Tlymphocytes. Patients with SCID are susceptible to infection with bacteria and viruses as there is a complete defect in adaptive immunity. 4. ADA (adenosine deaminase) and PNP (purine nucleotide phosphoryl ase) deficiency cause accumulation of nucleotide metabolites that are toxic to developing T -cells resulting in T-cell and B-cell deficiencies and a SCID phenotype 5. In this type of SCID. Patients with mutations resulting in non -functional Rag fail to develop both B . 9 and 15). SCID can be due to an autosomally inherited defect in DNA repair analogous to the genetic defect in the SCID mouse strain Teaching Notes for Question 147 Theme: Severe combined immunod eficiency Patients with SCID always have deficient T -cells but may or may not have absent B -cells. B-cell development is also affected to a lesser extent. Humoral immunodeficiency occurs because of a lack of T-cell help.SCIDs are du e to adenosine deaminase (ADA) deficiency. ADA and PNP are enzymes which catalyze the metabolism of purines via the uric acid pathway.SCID include mutations of DNA-dependent protein kinase (DNA-PK . Can easily distinguish primary colours by name 3. B-cells are present (T -B+ SCID). Functional Rag proteins are required for initiation of recombinatorial rearrangement of the genes encoding the antigen -specific receptors of both T.PEDIATRICS EXAMINATION REVIEW 3. In X linked SCID there is a mutation in the gene encoding the common gamma ch ain – a chain shared by receptors for a number of interleukin cytokines -(IL-2. Several defects can lead to the phenotype of severe combined immunodeficiency.an enzyme involved in DNA repair) and lack of expression of MHC class II or MHC class I molecules (bare lymphocyte syndrome). or RAG -1 or –2 deficiency. Should imitate observed actions 5. T-B. There is thus impaired T -cell differentiation as the interleukins are important T -cell growth factors. Deficiency of either of these enzymes leads to the accumulation of toxic metabolites which severely affect developing T-cells.

101 . 40% of patients with Turner’s Syndrome have coarctation and -or aortic stenosis. Perianal skin tags 5.normal developmental milestones Gross motor development: * walks well with feet only slightly apart . ASD’s account for 40% of all CHD 3. Hypopigmented spots on the lips suggestive of Peutz -Jeghers syndrome 3. a vasculitis that can cause GI bleeding. VSD’s account for 70% of all CHD.PEDIATRICS EXAMINATION REVIEW 18-month-old child . 50% of patients with trisomy 21 and 40% of those with Turner’s syndrome. Webbed neck and low hairline 4. ASD (secundum) is the second most common defect and accounts for 6 -8% of all lesions. Approx.Social behaviour * feeding . such as Marfan’s or Noonan’s. Approx. 151-The following are clues to the cause of an upper gastrointestinal (GI) bleed: 1.can use spoon without rotating it * drinks from a cup * may be dry by day (Forfar and Arneil's textbook of Paediatrics) 150-The following statements concerning congenital heart disease (CHD) are true: 1. 13% of patients with a chromosomal defect have associated CHD – heart disease is found in over 90% of patients with trisomy 18.Speech and language * 6-20 recognisable words * names 1-2 simple objects . Overall. Haematochezia due to peptic ulceration Teaching Notes for Question 151 Theme: Gastrointestinal bleeding There are many causes of GI bleeds and some have clues to their origin. but are bottom shuffling * pulls a toy as he walks * runs stiffly * climbs stairs holding on * throws a ball without falling Fine motor development: * tower of three cubes * scribbles * turns pages of a book two or three at a time * can use a spoon without rotation * can take off gloves and socks and unzip fasteners Personal and social development: . 80% of children with Down’s Syndrome have CHD 5. Approx. Approximately 3% of patients with congenital heart disease have an identifiable gene defect. Purpuric rash suggestive of Henoch-Schonlein purpura (HSP) 2. hair. 2. eyes on request . 13% of neonates with chromosomal abnormalities have associated CHD 4.some children are not walking yet.General understanding * points to own or doll’s nose.2-3 parts of the body * imitative play * points to named pictures . These include t he rash of HSP. Teaching Notes for Question 150 Theme: Congenital heart disease VSDs account for 25% of all congenital heart disease and are the most common anomaly.

152-The muscles of the soft palate are: 1. Gestational diabetes 102 . Hyoglossus 5. cervical ribs. Heterochromia refers to different pigmenta tion of the iris compared to the normal eye and is a feature of congenital Horner’s.PEDIATRICS EXAMINATION REVIEW Peutz-Jeghers syndrome .a syndrome manifesting with multiple intestinal polyps is associated with hyperpigmented spots on the lips. Palatoglossus 3. miosis and anhydrosis and is caused by interrupti on of the sympathetic supply to the eye. Palatopharyngeus Teaching Notes for Question 152 Theme: Soft palate Hyoglossus is an extrinsic muscle of the tongue. Tensor veli palatini 2. Perianal skin tags are found in inflammatory bowel disease. demyelination c) T1: apical carcinoma of lung (Pancoast syndrome). demyelination. Levator veli palatini 4. tumour. Haematochezia suggests a lower GI bleed. The pathway starts in the ipsilateral hypothalamus and has three neurones with synapses in the lateral grey of the spinal cord at T1 and superior cervical sympathetic ganglion. Causes depend on the level of the lesion: a) Brainstem: tumour. Heterochromia 5. Loss of sweating may not occur if the lesion is distal to the superior sympathetic ganglion. Acromegaly 3. Ipsilateral ptosis 2. 154-An abnormal response to oral glucose loading is seen in: 1. neck surgery e) Internal carotid: dissection. Partial gastrectomy 4. Contralateral mydriasis 3. jugular foramen syndromes All the features of Horner’s are ipsilateral. The affected eyeball often seems sunken (enophthalmos) owing to slight elevation of the lower eyelid. infarction b) Cervical cord: syringomyelia. A webbed neck and low hairline would indicate Turner Syndrome wh ich is associated with GI vascular malformations. 153-Horner’s syndrome invariably produces : 1. Wilson's disease 5. Malabsorption 2. Loss of sweating over the ipsilateral face 4. Exophthalmos Teaching Notes for Question 153 Theme: Features of Horner's syndrome Horner’s syndrome consists of ptosis. carotid body tumour. trauma d) Cervical sympathetic chain:thyroid carcinoma.

Hypoventilation is induced by metabolic alkalosis to raise PCO2 and retain acid in order to bring pH back into the normal range. 155-With regard to the visual pathways: 1. 155-Metabolic influences on pH 2: Which of the following statements are true? 1. If incomplete. Metabolic alkalosis can be induced by vomiting from the stomach. 156-The O2-Hb dissociation curve is displaced to the right in: 1. In Acromegaly. 2. 3. there is a paradoxical rise in GH levels. A pituitary adenoma may cause a bitemporal hemianopia Teaching Notes for Question 155 Theme: Visual pathway The visual pathway runs from the eye to the occipital cortex. or lower quadrantinopia if in the parietal region. Partial gastrectomy produces a lag response. in addition there will be enlargement of the blind spot. A lesion of the optic chiasma will cause a homonymous hemianopia 2. A lesion of the left occipital lobe will resu lt in a right homonymous hemianopia 3. Methemoglobinemia 3. In Wilson’s disease there is an exaggerated rise in GH. Metabolic alkalosis is the reduction in H+ concentration by any means other than decreased PCO2. 5. An elevated glucose 2 hours after loading suggests diabetes. The light reflex gives an indication of optic nerve function and will. 4. Increased central chemoreceptor firing rates decrease ventilation rates. Teaching Notes for Question 154 Theme: Metabolic alkalosis Increased peripheral chemoreceptor firing rates (but not central) increases ventilation rates. Increased lactic acid production 4. Ventilation is reflexly depressed by metabolic alkalosis. due to compression by pituitary tumors) result in bitemporal hemianopia. Hyperventilation induced by metabolic alkalosis returns arterial pH to the normal pre -set points.3 DPG 103 . Chiasmal lesions (e. therefore. Increased 2. be abnormal in lesions of the optic nerve. Lesions of the optic nerve cause an enlargement of the blind spot 5. Fetal Hb 2. The pupillary light reflex is preserved in lesions of the optic nerve 4.g. Chronic hypoxia due to cyanotic conditions 5. a lesion of the optic radiation with result in a contralateral upper quandrantinopia if in the temporal region. A lesion of the optic tract/radiation or occipital cortex will result in a contralateral homonymous hemianopia.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 154 Theme: Abnormal glucose tolerance test No rise in glucose and so no rise in growth hormone or insulin levels are typical of malabsorption.

blood should be given with caution: transfusion reactions are more likely. It can be given IM for protection of susceptible contacts against hepatitis A. where there is an increase in 2. Urine dipstick is a useful investigation 4. Renal blood flow = plasma flow/(1 . * Increase in CO2 production due to increased metabolism * Increase in H+ * Hyperthermia * Chronic hypoxic states/anemia.HCT) 4. First line treatment is imipramine 5. IgA deficiency 3. Metachromatic leukodystrophy is an autosomal recessive lysosomal storage disorder. Metachromatic leukodystrophy Teaching Notes for Question 157 Theme: Intravenous immunoglobulin (IVIG). Intravenously. The filtration fraction is the ratio of GFR to renal blood flow 2. it is used as antibody replacement for patients with hypogammaglobulinaemia or agammaglobulinaemia but it is contraindicated in IgA deficiency because of the possibility of anti-IgA antibodies in patients with complete deficiency. IVIG is now preferred to plasma exchange in the treatment of Guillain-Barre syndrome. IVIG and steroids can be used to treat ITP. 158-In the kidney 1. O2 is easily given up and this will be beneficial in conditions when the body is working hard.2 3. High dose IVIG (2g/kg) is used to treat Kawasaki disease. Idiopathic thrombocytopenia purpura 4. Guillain-Barre syndrome 2. GFR can be measured using a thermodilution technique Teaching Notes for Question 158 Theme: Glomerular filtration The filtration fraction is the ratio of GFR to renal plasma flow 159-In enuresis :1. Kawasaki disease 5. Conditioning is usually successful 104 . 5% of 5 year old children are affected 3. Normal filtration fraction is 0.1 -0. Human normal immunoglobulin is prepared from pools of at least a thousand donations of human plasma and contains antibody to common viruses. measles and rubella.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 156 Theme: Oxygen hemoglobin dissociation curve When shifted to the right. For the same reason. In a 3 year old investigations must be carried out 2. GFR can be measured using Doppler ultrasound 5.3 DPG 157-Intravenous immunoglobulin (IVIG) is used in the treatment of: 1.

Boys more common than girls. Cushing’s syndrome 2. Carcinoid syndrome Teaching Notes for Question 160 Theme: Endocrine causes of diarrhoea Addison’s disease and thyrotoxicosis can both present as diarrhoea. Genetic factors involved. Mainstay is behavioural conditioning methods . However complex partial epilepsy can involve generalised seizures such as absence and tonic-clonic seizures. Occurs in 10-15% of 5 year olds and 3-5% of 10 year olds. Carcinoid results in diarrhoea and facial flushing once metastasis to the liver has occurred. They are focal frontotemporal seizures. The tumours secrete 5 HT. desmopressin -tablets or nasal spray. and are commonly sited in the terminal ileum or appendix. Hyperparathyroidism 3. EEG changes disappear in sleep 5.bell and pad. Carcinoids make up 10% of all small bowel tumours. Hyperventilation often provokes a complex partial seizure 4. Right sided cardiac valve lesions can also occur. dermatitis and dementia. 161-Regarding complex partial epilepsy:1. Medication: tricyclic antidepressants. Primary enuresis. Pamidronate is a recognised treatment in children with this condition 105 . Night time wetting more common than daytime wetting. histamine and bradykinins. Thyrotoxicosis 4. Carbamazepine and sodium valproate are effective treatments for complex partial seizures. Developmental delay in neurological maturation. Blue sclera is diagnostic 3. Sleep often enhances EEG abnormalities. IDDM or neurological disorder. Consciousness is altered 3. There is an increased incidence of osteosarcoma 2. star charts.when child has never been dry. Diarrhoea can complicate diabetes mellitus due to the aut onomic neuropathy. 160-Endocrine disorders that result in diarrhoea include: 1. Primary hyperaldosteronism 5. Must rule out organic causes first eg UTI. not complex partial seizures. Absence seizures and generalised tonic -clonic seizures may occur 2. Diagnosis is by urinary 5HIAA. include altered consciousness. resection of the primary or octreotide to reduce symptoms. by definition.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 159 Theme: Enuresis Enuresis: is the involuntary voiding of urine by day and/or night. Ethosuximide is only effective in absence seizures. The diversion of typtophan from the niacin synthesis pathway can result in a pellagra à diarrhoea. Ethosuximide is an affective treatment Teaching Notes for Question 161 Theme: Complex partial seizures Complex partial seizures. Treatment: simple methods such as reducing fluid intake prior to going to bed. 162-In osteogenesis imperfecta 1. Treatment is by embolisation of the secondaries. Hyperventilation provokes absence seizures.

profuse sweating. Ehlers -Danlos and normal infants.5 cm2) is usually associated with normal RV pressures. recurrent chest infections and cardiac failure in infancy. poor growth. In non restrictive VSDs (>1 cm2). 164-Neostigmine: 1. Can present with heart failure in the neonatal period 2. * It is metabolized by plasma and hepatic esterases and is excreted predominantly in the urine. Cardiac bypass is not necessary for surgical correction Teaching Notes for Question 163 Theme: Ventricular septal defects VSD is the most common cardiac malformation accounting for 25% of all congenital heart disease. Tricuspid atresia is a recognised association 5. In the tetralogy of Fallot. biopsy is rarely done. Small VSDs are usually asymptomatic with a harsh pansystolic murmur at the LLSE – in neonates. As the PVR continues to fall in the first few weeks after birth. but also approx 30% in the bile. 163-Ventricular septal defects: 1.The majority of defects are in the membranous septum. Crosses the placenta Teaching Notes for Question 164 Theme: Anticholinesterases * Neostigmine also inhib its pseudocholinesterase and so can prolong the duration of action of suxamethonium. RV and LV pressures equalise. Is metabolized by plasma esterases 5. Has an oral bioavailability of 50% 4.PEDIATRICS EXAMINATION REVIEW 4. Although not a licenced treatment. The degree of left -to-right shunt is determined by the size of the defect and the pulmonary vascular resistance as compared to the SVR. the size of the left-to-right shunt increases and clinical symptoms become ap parent – dyspnoea. May regress by incorporation of the tricuspid leaflet tissue 4. it does not cross the BBB or the placenta owing to its highly ionized state at physiological pH and also its quaternary amine s tructure. A restrictive VSD (<0. Prolongs the duration of action of suxamethonium 3. Hearing abnormalities are common 5. OI is a fairly easy clinical diagnosis to make. posteroinferiorly. Patients with a large VSD often have a limited left-to -right shunt immediately after birth as the pulmonary resistance may remain higher than normal. the murmur may be inaudible du ring the first few days of life as the pulmonary pressures are high. Has been used in the treatment of SVT 2. and anterior to the septal leaflet of the tricuspid valve. feeding difficulties. and is associated with pulmonary stenosis. Diagnosis is usually made by bone biopsy Teaching Notes for Question 162 Theme: Osteogenesis imperfecta OI results from muta tion in Type I procollagen gene. Blue sclera is also found in Marfan’s. the defect lies between the crista supraventricularis and the papillary muscle of the conus.2%. 106 . * Its oral bioavailability is only 1 . bisphosphonates have been shown in series to improve BMD and reduce fractures. Results in wide splitting of the second heart sound 3.

Inhibit gluconeogenesis. Molecules consist of 21 carbon atoms. 167-Glucocorticoids: 1. stepping reflex (11 months). * Fabry’s disease. Cause retention of sodium. Galactosemia 3. A newborn baby from 32 weeks gestation will turn towards a light Teaching Notes for Question 166 Theme: neuro -developmental assessment Most primitive reflexes disappear by 6 months of age. Deep tendon reflex es are difficult to elicit at birth 4. Septicemia Teaching Notes for Question 165 Theme: Cataracts. systemic diseases A cataract is simply any loss of the transparency of the lens of the eye. * Diabetes mellitus. Muscular dystrophy 2. The moro reflex consists of arm extension followed by flexion with extension of the fingers. An extensor Babinski response is normal in the first year 5. Exceptions are the plantar reflex (9 months). and usually flexion of the t highs at the hips. 3. The list of systemic associations with cataracts is too long to discuss in full. A newborn baby from 32 weeks gestation with a normally functioning visual cortex will turn the head towards a light.PEDIATRICS EXAMINATION REVIEW 165-Recognized associations of systemic conditions and cataracts include: 1. asymmetric tonic neck reflex (9 months). but important associations include: * Myotonic dystrophy (not muscular dystrophy! ). 107 . Hyperthyroidism 4. Deep tendon reflexes are present and usually brisk at birth. * Toxoplasmosis and * CMV. * Congenital rubella. * Down’s syndrome. 166-The following statements are true: 1. 2. * Galactosemia. * Atopy. Reduce lymphocyte counts. A moro reflex with the fists remaining clenched is normal. Are secreted from the zona fasciculata within the adrenal cortex. * Wilson’s disease. 2. The plantar grasp persists longer than the palmar grasp 3. 4. 5. Relapsing polychondritis 5. The Babinski response is usually extensor until the infant begins to walk (usually from 1 year). A moro reflex with the fists remaining clenched is abnormal.

Thyroid peroxidase catalyses the formation of thyr oxine. Poor appetite is a common side affect of methylphenidate (Ritalin) Teaching Notes for Question 168 Theme: Attention deficit hyperactivity disorder Treatment of attention deficit hyperactivity disorder: stimulant medication methylphenidate (Ritalin). 2.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 167 Theme: Glucocorticoids The glucocorticoids consist of cortisol and corticosterone. TRH is released by the anterior pituitary. Behavioural therapy is never useful 2. thrombocytopenia. Iodination of the tyrosine residues of thyroglobulin leads to the prod uction of T3 and T4. Side effects include difficulty sleeping. Parenting techniques can be taught 5. There is a slight mineralocorticoid effect. and also an anti inflammatory effect. Iodine is taken up by secondary active transport. loss of appetite. dexamphetamine. coupled with sodium. Pharmacological intervention includes the use of stimulant drugs such as amphetamines 3. ab dominal pain. T4 is released in greater quantities than T3. Outcome: symptoms tend to lessen as child gets older. Primary ciliary dyskinesia 3. Pyloric stenosis Teaching Notes for Question 170 Theme: Autosomal recessive diseases Diseases that are inherited in an autosomal recessive manner are usually those in which 108 . glucose and fat metabolism. headache. Iodine is taken up passively by the thyroid gland. Teaching Notes for Question 169 Theme: Thyroid physiology TRH is secreted by the hypothalamus. Behavioural modification. 168-In the treatment of attention defici t hyperactivity disorder :1. The main effects are in protein. Congenital adrenal hyperplasia 5. It stimulates release of TSH from the anterior pituitary. Methylphenidate (Ritalin) should not be given later than 4pm 4. T4 is released bound to thryroglobulin. Not to be taken after 4pm. 170-The following diseases are inherited in an autosomal recessive manner: 1. 169-Concerning the synthesis of thyroid hormones: 1. Subsequently it is oxidised and coupled to thyroglobulin. 4. Insulin dependant diabetes 2. 5. which in turn u p-regulates synthesis of hormones by the thyroid gland. 3. which are cleaved from the parent molecule prior to release. parenting techniques. Batten's disease 4.

and hence may cause a reflex bradycardia. Increased oxygen concentrations 2. although coronary perfusion may be increased. The ductus arteriosus closes in response to decreased oxygen concentrations 5.Batten Disease is an AR disorder als o known as SpielmeyerVogt-Sjogren -Batten Disease. As pulmonary pressures fall after birth. The umbilical artery is a branch of the common iliac artery Teaching Notes for Question 172 Theme: Cardio -pulmonary changes at birth Functional closure of the ductus arteriosus occurs soon after birth but anatomical closure can take upto one week. It causes an increase in metabolic rate. 171-Noradrenaline 1. Prostaglandin E2 keeps the ductus open. Is a neurotransmitter at the pre-ganglionic sympathetic nervous system 2. May cause a reflex bradycardia when given by infusion 4. It acts predominantly via the beta adrenoceptor Teaching Notes for Question 171 Theme: Catecholamines Noradrenaline is a catecholamine produced by the adrenal medulla. it is the most common form of a group of disorders called Neuronal Ceroid Lipofuscinoses (or NCLs). Is entirely retroperitoneal 2. Decreased prostaglandin levels 3. Blood flow in the ductus arteriosus continues from right to left until its closure 3. leading to a rise in blood pressure. and progressive loss of sight and motor skills. It causes vasoconstriction. Drop in pulmonary artery pressures N. Factors influencing closure of the ductus include: 1. in the heart. Decrease in pulmonary artery resistance following closure of the ductus arteriosus 4. Causes a rise in metabolic rate 5. 173-The pancreas: 1. Functional closure of the foramen ovale in the first 24 hours 2. Affected children suffer progressive mental impairment. dynein. Is crossed by the attac hment of the transverse mesocolon 109 . Is supplied by branches of the coeliac artery alone 3. worsening seizures. It also acts as a neurotransmitter in the post-ganglionic sympathetic neurons and parts of the brain.B. blood flow in the ductus is reversed ie from left to right. It activates the baroreceptor reflex. myocardial oxygen consumption will also be increased. Primary ciliary dyskinesia (PCD) is an exception since at least one of the genes encodes a structural protein of the cilia. Its actions are mediated mostly through the alpha -adrenoceptor. though it does have some action at the beta-adrenoceptor. hence. 172-In a healthy baby the transition from fetal to neonatal circulation involves: 1. Is produced by the adrenal cortex 3. The inh eritance of pyloric stenosis and insulin dependant diabetes is multifactorial although it has been proposed that pyloric stenosis is inherited as autosomal dominant with reduced penetrance on a multifactorial background.PEDIATRICS EXAMINATION REVIEW there is a biochemical defect. The umbilical artery is a branch of the internal iliac artery.

affecting the levels of IgA (A1. hyperactivity in childhood.and post.jejunal biopsy should be performed. May delay diagnosis of coeliac disease 4. GI and GU infections.PEDIATRICS EXAMINATION REVIEW 4. Is usually asymptomatic 3. 174-IgA deficiency:1. but occasionally there is an increased incidence of respiratory. bu t also IgM. Spontaneous recovery is a recognised feature 5. anaphylaxis. Intra uterine growth retardation 5. irritability in neonatal period. pre . Is seen in Kartagener's syndrome 2. In selective IgA deficiency there is an increased incidence of cows' milk allergy.M+A) iv) Ataxia telangiectasia (IgA+E) v) Bloom's syndrome (IgA+M) vi) Kartagener's syndrome (IgA) vii)Selective IgA deficiency (IgA)Selective IgA deficie ncy is common (1:350) but the incidence is not universal worldwide (rarer in Japan 1:1. Hypotonia Teaching Notes for Question175 Theme: Fetal alcohol syndrome The incidence of fetal alcohol syndrome is thought to be as high as one in 1000 babies born in UK. A2 & secretory forms). Characteristic features include IUGR. microcephaly. Hyperactivity in childhood 4. Most people are completely asymptomatic. facial 110 . Increasingly the diagnosis is been made following investigation of FTT where the diagnosis of coeliac disease is made based on serology (antigliadin & endomyesial IgA) and therefore it is worth bearing in mind in a negative coeliac screen where malabsorption seems likely and other conditions are similarly ruled out. 175-Abnormalities associated with foetal alcohol syndrome include: 1. hypotonia.. autoimmune disease and malignancy.800). Up to 32% of infants born to heavy drinkers demonstrate congenital malformations. In transient hypogammaglobulinemia of infancy following the erosion of maternal passive immunity there may be a delay of up to 36 months before full humoral immunity is restored. moderate to severe mental retardation. The levels of IgG & A are predominantly affected. The greater the intake of alcohol the more severe the signs in the neonate. Alternatively. Short palpebral fissures 2. Is crossed by the left renal vein 5. Affects both serum and secretory IgA levels Teaching Notes for Question 174 Theme: IgA deficiency IgA deficiency may be seen in: i) CVID (all Ig groups) ii) Bruton's agammaglobulinemia (all Ig groups) iii)Transient hypogammaglobulinemia of infancy (IgG. Maxillary hyperplasia 3. Is related to the common bile duct posteriorly Teaching Notes for Question 173 Theme: Abdomen Tail of pancreas lies intraperitoneally and can be damaged easily during splenectomy when the splenic vessels are clamped.

PEDIATRICS EXAMINATION REVIEW abnormalities such as short palpabral fissures. micrognathia. 2. Deficiency in glucose-6-phosphate dehydrogenase results in impaired motility of leukocytes 5. maxillary hypoplasia. -dehydration -trauma -metabolic disorders eg MELAS. and thin upper lip. Haemoglobin SS 5. which are necessary for protein synthesis. 3. 176-The seventh cranial nerve: 1. The molecular defect in X -linked hyper IgM syndrome is in the expression of CD40 on B -cells 111 . Teaching Notes for Question 176 Theme: The seventh cranial nerve The difference between the pattern of facial weakness seen in lower an d upper motor neurone lesions is explained by the presence of bilateral cortical representation. cardiac defects (mainly septal defects). Activated protein C resistence 3. The defect in Chediak-Higashi syndrome is in T-cell activation 4. Nephrotic syndrome 2. Receives taste fibres from the palate. 4. activated protein C resistence. Receives taste fibres from the anterior 2/3rd of the tongue via the greater petrosal nerve. When lesioned below the level of the stylomastoid foramen will cause hyperacusis. Due to arterial occlusion. Systemic lupus erythematosis 4. 177-Strokes in childhood are associated with : 1. Moya moya disease Teaching Notes for Question 177 Theme: Cerebrovascular Disease Strokes are rare in childhood. There is some evidence to suggest that alcohol may interfere with transfer of essential amino acids and zinc. venous thrombosis. Causes: -sickle cell disease -arteriovenous malformations and vascular dysplasia eg moya moya disease -vasculitides eg SLE. The commonest inherited form of severe combined immune deficiency is x -linked 2. Herpetic vesicles may be seen in the ear canal in patients with geniculate zoster (Ramsay Hunt syndrome): this is why the sensory supply to this area is important. PAN -coagulopathies eg AT III. The incidence of lymphoid malignancy is increased in ataxia telangiec tasia 3. The seventh nerve receives taste fibres from the palate (via the greater petrosal nerve) and the anterior 2/3rd of the tongue (via the chorda tympani and the lingual branch of the fifth). Has a sensory supply in the ear canal. epicanthic folds. homocysteinuria 178-The following statement(s) concerning inherited immunodeficiency s yndromes are true: 1. 5. protein C and S deficiency. Has bilateral cortical representation.

HBV and HCV. low IgE. Malaria. HIV. 2. Other clinical features include partial oculocutaneous albinism and abnormal platelet function. the commonest inherited form is X-linked. 180-Leptospirosis:1. 179-The following diseases may be transmitted by needlestick injury: 1. characterised by giant granules in phagocytes due to a genetic defect in intracellular vesicle formation and hence impaired intracellular killing following phagocytosis. Tuberculosis. Teaching Notes for Question 179 Theme: Needlestick injury Over 20 infections have been shown to be transmissible by needlestick injury. Clinically. Chediak -Higashi syndrome is an autosomal recessive disorder. Patients have impaired cell mediated immunity. Hepatitis B. Deficiency of either of these enzymes leads to the accumulation of toxic metabolites which severely affect lymphocyte production and function. Hepatitis C. This leads to impairment in immunoglobulin isotype switching and germinal ce ntre formation as CD40/CD40L interaction is needed for these processes. Lack of expression of MHC class II or MHC class I molecules also cause severe immunodeficiency.a process important for intracellular killing by phagocytes. Cytolytic T -cell-mediated killing is normal. but NK killing is impaired. Ataxia telangiectasia is due to defects in ATM. use of safer needles. May be diagnosed on urine culture 3. sharps bins and so on. Is treated with tetracycline 112 . X -linked hyper IgM syndrome is due to the lack of CD40 ligand (CD40L) expression on activated T -cells. Other known genetic defects that lead to severe immunodeficiency include mutations of DNA-dependent protein kinase (DNA-PK) and recombination activating genes (RAG). Lymphoid malignancies as well as carcinomas occur with with increased frequency in these patients. Prevention is key. The commonest autosomally inherited forms are adenosine deaminase and purine nucleotide phosphorylase deficiency. with attendance at Occupational Health. ADA and PNP are enzymes which catalyze the metabolism of purines via the uric acid pathway. by sharps awareness training. 4. Management of an injury should include encouraging bleeding. Humoral immunodeficiency occurs because of a lack of T-cell help. The enzyme also functions in the respiratory burst . washing and disinfecting the wound and reporting the incident. The patient may be approached for consent for testing for HIV. Serum IgM levels are high with deficiency of IgG and IgA. these patients are particularly susceptible to opportunistic lung pathogens such as Pneumocystis carinii.the clinical picture resembles a mild form of chronic granulomatous disease. Most recover within two weeks without treatment 2. 3. G6PDH is an important enzyme in the hexose monophosphate shunt. a protein that is important in DNA repair. Complete G6PDH deficiency results in susceptibility to chronic bacterial and fungal infection . There is thus impaired T -cell differentiation as the interleukins are important T -cell growth and differentiati on factors. There is a mutation in the gene encoding the common gamma chain – a chain shared by receptors for a number of interleukin cytokines. IgA and IgG2 and are prone to respiratory infections and skin infections. This pathway maintains glutathione in a reduced state protectin g the red cell membrane from oxidant stress .PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 178 Theme: Inherited immunodeficiency syndromes Several defects can lead to the phenotype of severe combined immunodeficiency. 5.

via contact sports (L hardjo). the organism gains entry via mucous membranes or cu ts. K+ 2. Is a valveless system 2. May be complicated by thrombotic thrombocytopenic purpurea 5. Maintains a uni-directional flow of lymph towards the thoracic ducts 3. Sugar Teaching Notes for Question 182 Theme: Cerebrospinal fluid CSF has a very low plasma concentration because large protein molecules cannot cross the blood-brain barrier. and if treatment is prompt. Osmolarity 4. Protein 5. * The total lymph drainage for an adult. A minority of patients develop meningitis. Has a total lymph drainage of 6-8 litres of fluid/24 hours from inter stitial fluid 5. The disease affects predominantly men. The thoracic ducts drain into the venous circulation at the junction of the sub -clavian veins and internal jugular veins 4. or serologi cally.icterohaemorrhagiae). from the interstitial fluid. * Distension by the lymphatic fluid causes contraction of this smooth muscle. Is more common around the New Forest Teaching Notes for Question 180 Theme: Leptospirosis Diagnosis relys on demonstration of leptospira in blood and CSF. The Na+ concentration is roughly the same or slightly more in CSF and the K+ concentration is slightly less in CSF. 1.PEDIATRICS EXAMINATION REVIEW 4. and is commonly acquired through occupational contact (L hardjo from cattle). Contains lymph draining from the liver and intestine with a protein concentration of approximately 40 -60 g/l Teaching Notes for Question 181 Theme: Lymphatic system * Lymph flows in single layered lymphatic capillaries into lymphatic vessels that contain smooth muscle. The CSF sugar content is about two third than that of blood. is about 2 -4 liters/24 hours. cholestatic jaundice and renal failure (Weil's disease). 181-The lymphatic system: 1. Treatment: penicillin. or exposure to rat urine (L. Antacids and prednisolone 113 . 182-The following have a much lower concentration in th e cerebrospinal fluid (CSF) than in the cerebral capillary blood. Na+ 3. * Valves then direct flow towards the thoracic ducts in a uni -directional fashion. the renal failure of Weil’s disease can recover. 183-Co-administration of the following pairs of drugs results in reduced absorption of the latter:1.

4. Cholestyramine and warfarin 3. Leads to increased levels of plasma angiotensinogen 2. May be a sign of cerebral palsy Teaching Notes for Question 184 Theme: Abnormal Gait Pathological causes of toe walking are i) neuromuscular disorders e.PEDIATRICS EXAMINATION REVIEW 2. the aldosterone level is elevated whilst the renin level is low or undetec table. Spinal cord imaging may be indicated 3.g. Is more common in females. iron and prednisolone. 5. Metoclopromide increases gastric emptying and is used in combination with analgesics in migraine to enhance their absorption. Teaching Notes for Question 185 Theme: Conn's syndrome Conn's syndrome is common in males and is associated with hypertrophy of zona glomerulosa. Toe walking may be normal but this is a diagnosis of exclusion.g. aldosterone and renin (which play a role in stimulating aldosterone production). Its other name is primary hyperaldosteronism. Is always abnormal 2. 114 . 185-Conn's syndrome: 1. ii) upper motor neurone lesions (i. 2. 3. muscular dystrophies or myopathies. leading to excess aldosterone production. Toe walking may be a feature of diplegic cerebral palsy. The best method of diagnos is is to measure the blood levels of two hormones. Duchenne muscular (where initially due to proximal weakness and pelvic instability shifting the centre of gravity forward and later to tightness of the tendo a chilles) dystrophy. The antimuscarinic effect of tricyclics reduces gastric emptying and delays absorption. Causes muscle weakness. Is due to atrophy of zona glomerulosa. Serum creatine kinase should be measured 4. Metoclopramide and paracetamol Teaching Notes for Question 183 Theme: Absorption Aluminium and magnesium (in antacids) form insoluble complexes with tetracyclines. Sucralfate and phenytoin 4.e. In Conn's syndrome (primary hyperaldosteronism). Leads to sodium retention. 184-Toe-walking in an 18 month old child : 1. Creatine kinase is a muscle enzyme and is increased in conditions with increased muscle turnover e. in brain and spinal cord) due to extensor hypertonus and tightness of tendo achilles. Amitriptyline and digoxin 5. Does not need to be investigated in a girl 5.

115 . Have receptors that recognise bacterial cell wall constituents 2. Is associated with Jaccoud's arthropathy 3. These cells initiate and amplify the adaptive immune response by producing pro -inflammatory cytokines such as IL1. Phagocytes of the innate immune system recognise com mon motifs on invading pathogens using cell surface pattern recognition receptors. Hemachromatosis is associated with HLA A3. Release cytokines wich affect the adaptive immune system 4. Require clonal expansion to mount an effective response 3. Binding of these receptors to their ligands enhances phagocytosis of antigen . Are important for the resolution of inflammation 5. Is associated with HLA B8 5. Is more common in men 4. 188-With regards to aniridia:1. Jaccoud's arthropathy is non erosive and associated with SLE. Is associated with vitamin C deficiency 2.a process termed opsonisation.B14 rather than B8. IL6 and TNF. These cells phagocytose foreign particles and microbes. 2. Treatment is with venesection and desferrioxamine (one molecule of desferrioxime binds one molecule of iron). Is associated with a gene on chromosome 13 Teaching Notes for Question 186 Theme: Haemochromatosis Hemochromatosis is autosomal recessive and is carried on the short arm of chromosome 6.DR3. as does chondrocalcinosis in 50 percent of cases. Phagocytic cells enter tissues by rolling on selectins and binding via integrins to ICAM (and similar molecules). They mount a rapid response that does not require clonal expansion. Two thirds of the cases are sporadic. Initiate firm attachment by binding to ICAM on the endothelium Teaching Notes for Question 187 Theme: Phagocytic Cells The major phagocytic cells of the immune response are macrophages and neutrophils. Erosive arthritis affecting the hip/knee/wrist and 2 nd and 3rd MCP joints occurs. There is an associated Vitamin C deficiency. W ilson's disease is associated with a locus is on chromosome 13. They are also important in clearing apoptotic cells and debris in the resolution phase. It is usually bilateral. 187-Phagocytic Cells :1. Internalised microbes are killed by phagocytes which secrete reactive oxygen intermediates and possess potent microbicidal properties. They transmigrate by binding to PECAM and similar adhesion molecules. Menstruation protects homozygous women from the effects of iron overload. Phagocytic cells have surface receptors that recognise the Fc portion of immunoglobulin as well as complement components. acute rheumatic fever and hypocomplementaemic vasculitis.PEDIATRICS EXAMINATION REVIEW 186-Haemochromatosis:1.

PEDIATRICS EXAMINATION REVIEW

3. Vision is commonly decreased. 4. It is associated with partial deletion of long arm of chromosome 11. 5. Wilm's tumour is associated with familial aniridia.

Teaching Notes for Question 188 Theme: Aniridia In aniridia, the iris tissue is hypoplastic, not usually absent. Two thirds of the cases are dominantly inherited and 1/3rd are sporadic. It is bilateral in 98% of cases. Macular and optic nerve hypoplasia are common and leads to visual impairment. Corneal and lens abnormalities may occur and glaucoma occurs in 75% of patients with aniridia. Wilm's tumour ma y occur in 1/5th of patients with sporadic aniridia. There is an association of anirida, genitourinary anomalies, mental retardation and a partial deletion of the short arm of chromosome 11.
189-Immune Complex deposition in the kidney occurs in: 1. Goodpasture's disease 2. Infective endocarditis 3. Systemic lupus 4. Polyarteritis nodosa 5. Plasmodium malariae infection

Teaching Notes for Question 189 Theme: Immune complex deposition In Goodpasture's disease, circulating antibodies bind to the antigen in the glomerular basement membrane and the alveolar membrane. There are no circulating immune complexes. Immune complexes are also found in SLE, serum sickness, Takayasu’s arteritis, hepatitis B antigenemia, etc
190-Regarding oxygen measurement: 1. Red light absorbs more oxyhemoglobin than deoxyhemoglobin 2. The isobestic point will vary with hemoglobin concentration 3. The readings from pulse oximeters are affected by anemia 4. The clark electrode uses a silver/silver chloride cathode 5. Water vapor will cause inaccurate readings from a paramagne tic analyzer

Teaching Notes for Question 190 Theme: Oxygen measurement * Red light absorbs, less oxyhemoglobin than deoxyhemoglobin, hence oxygenated blood looks red. * The isobestic point (where absorption of the two forms is the same) depends on hemoglobin concentration. * Pulse oximeters will not give accurate readings in states of vasoconstriction or venous congestion, high carbon monoxide levels, methemoglobinemia or lo w oxygen tensions. * Nail varnish/ stains on the skin or nail will also distort the readings.

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* Dark skin, jaundice or anemia will not affect the results. * The clark or polarographic electrode features a platinum cathode and Ag/AgCl anode in an electrolyte of KCl. * Water vapor tends to affect the readings of most gas analyzers.
191-The following syndromes/ associations are associated with congenital heart disease. 1. holt-Oram syndrome. 2. CHARG association. 3. Vacterl association. 4. Septo-optic dysplasia. 5. Goldenhar

Teaching Notes for Question 191 Theme: Congenital heart disease. Holt-Oram syndrom e is an autosomal dominant syndrome consisting of limb defects, thumb/radius/ulna hypoplasia associated with atrial & ventricular septal defect. CHARGE =Coloboma, heart defects (Tetralogy of fallot, coarctation of the aorta,and ventricular septal defect, c hoanal atresia), growth/mental retardation, genitourinary abnormalities, ear abnormalities including deafness. Cardiac defects include VSD & ASD’s, conotruncal malformations, PDA&tetralogy of fallot VACTERL=Vertebral, anal atresia, cardiac (VSD), tracheo -esophageal fistula,oesophageal atresia, renal,limb defects. The most common associated heart defects are VSD,ASD &Tetralogy of fallot. Less common defects are truncus arteriosus and transposition of the great arteries. Septo-optic dysplasia is a midline defect consisting of hypopituiterism and optic nerve hypoplasia with absence of the septum pellucidum, there may be midline defect of the heart (VSD, ASD). Goldenhar Syndrome is associated with VSD, PDA, and Coarctation of the aorta,and transposition of the great arteries, in addition they may be renal anomalies, neurological & musculoskeletal anomalies. .The cardinal features are anomalies of the face, ears &vertebrae.

192- Regarding myasthenia gravis in childhood: 1. The neonatal form is always due to maternal antibodies. 2. The diagnostic test is subcutaneous endophonium. 3. EMG shows decremental response to repetitive motor nerve stimulation. 4. Thyroid dysfunction is a recognised association. 5. The disease can be confined to ocular muscles.

Teaching Notes for Question 192 Theme: Myasthenia Gravis. Neonatal form is commonly due to maternal antibodies, but may also be an inherited channelopathy. In 40% of childhood myasthenia, the disease remains confined to the occular muscles. Generalised weakness is present at onset in 40% or in 20% develops within the year. 10% of juvenile cases have associated hyperthyroidism.

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193- The following statements regrd the treatment of asthma: 1. All patients should be on inhaled steroids. 2. High dose regular short acting bronchodilators should be tried before inhaled steroids. 3. Either long acting beta-2 agonists (LABAs) and low dose steroid or high dose inhaled steroid can be used to the same clinical effect. 4. Patient education is emphasized. 5. Leukotrene inhibitors are recommended in step 4.

Teaching Notes for Question 193 Theme: Treatment of asthma. Some patients with mild or exertional asthma can be controlled on short acting beta agonists alone. Opinion about what is exce ss short acting beta agonist use between cinicians however. 9 studies with serevent and fluticasone and one with eformoterol and budesonide confirm that steroid LABA combination is as effective inhaled steroid dose. In the new BTS/SIGN asthma guidelines LA BA therapy is recommended at step 3 before increasing inhaled steroid dose., with other therapies such as leukotriene inhibitors coming later in the treatment cascade.
194- Regarding child development: 1. Sense of taste is present from birth. 2. 25% of children are left handed. 3. Left handedness is more common in exprem babies without cerebral p alsy. 4. Left handed children are more likely to have un intentional injuries. 5. Prevalence rate of thumb-sucking in children are universal across races.

Teaching Notes for Question 194 Theme: Child development. Sense of taste is prersent from birth. Infants show prefe rence for sweet over salty or plain water. 7 -10% of children are left handed. This is a surprise as animals e.g. cats & dogs are equally left or right paired. Up to 25% of exprem babies without cerebral palsy are left handed but the reason is unclear. It is thought to be due to antecedent brain injury. Left handed children are more likely to have un intentional injuries as well as a higher likelihood of developmental disorders e.g.dyslexia and autism. Prevalence rates of thum -sucking in children vary from 4 5% of US children to 1% in eskimos. It tends to be less where children are carried by parents.
195-Persistant, conjugated hyperbilirubinaemia may be caused by: 1. Alpha-1-antitrypsin deficiency. 2. Hypothyroidism. 3. Haemolytic disease. 4. Cytomegalovirus infection. 5. Cystic fibrosis.

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Teaching Notes for Question 195 Theme: Neonatal jaundice. all cases need further investigation. Causes of conjugated hyperbilirubinaemia in a neonte are: 1)-Intrahepatic cholestasis -Infections. -Congenital infections (STORCH-syphlis, toxoplasma, rubella, cytomegalovirus, hepatitis, herpes virus infection) -Acquired infections- septicaemia, UTI. -Metabolic disorders –cystic fibrosis, alpha-1- antitrypsin deficiency, galactosaemia, fructosaemia, lysosomal storage disorders, peroxisomal disorders. -Endocrine disorders – hypothyroidism, hypopituitarism, hypoadrenalism. -Anatomical disorders – intrahepatic:bile duct hypoplasia. -Miscellaneous – idiopathic neonatal hepatitis, chromosomal abnormalities, trisomy 21, 18 and 13. 2)-Extrahepatic cholestasis, biliary atresia, choledochal cyst, spontaneous bile duct perforation, inspissat ed bile syndrome. Unconjugated neonatal jaundice is normally physiological and resolves spontaneously.
196-Complete transaction of the oculomotor nerve results in : 1. Ptosis. 2. Convergent squint. 3. Constriction of the pupil. 4. Loss of accommodation and light reflex. 5. Diplopia.

Teaching Notes for Question 196 Theme: Oculomotor nerve. The nucleus of the third nerve lies ventral to the aqueduct in the midbrain. Efferent fibres to four external ocular muscles (superior, inferior and medial recti, and inferior oblique), levator palpebrae superioris and sphincter pupillae (parasympathetic) enter the orbit thro ugh the superior orbital fissure \9from kumar and clark). A complete oculomotor palsy results in the affected eye being “displaced down and out” with a dilated pupil.
197-The following involve deficiencies in T -cell immunity: 1. Di George syndrome. 2. Wiskott-Aldrich syndrome. 3. Chronic granulomatous disease. 4. Bruton’s agammaglobulinaemia. 5. Chronic mucocutaneous candidiasis.

Teaching Notes for Question 197 Theme: Deficiencies in T -cell immunity. Di George syndrome and chronic mucocotaneous candidiasis are predominantly T cell defects. The underlying defect in Di George syndrome is failure of normal development of the 3 rd and 4th pharyngeal pouches leading to aplasia or hypoplasia of the thymus and associated disorders. T -cell numbers are reduced.

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120 .PEDIATRICS EXAMINATION REVIEW Chronic mucocutaneous candidiasis presents with persistant candidal infection of the nails. usually in expiration. May last for a few minutes to half an hour. 3. Breath Holding spells: Common in preschool children. All Ig levels are low and mature B -cells and plasma cells are present. frightened and inaccessible. Parental reassurance. T -cell numbers and function are normal. Drug treatments not thought to be particularly effective. Breath holding attacks cause cyanosis. not responding when spoken to. 198-In young children. Child eventually goes back to sleep. 1. Chronic myeloid leukaemia. Occur on waking from deep sleep . 4. Bruton’s agammaglobulinaemia is an X -linked recessive B-cell (humoral) defect due to mutation in the btk gene. Wiskott-Aldrich syndrome is associated with humoral and T-cell immunodeficiency. Follow crying which increases in intensity until breathing stops. 3. Chronic granulomatous disease is due to defective neutrophil killing. unlike nightmares that occur in REM or light sleep. Behavioural treatments best. Usually before the age of 2. Diabetes insipidus. skin and mucu s membranes. there is a minor epileptic seizure. 5. Teaching Notes for Question 198 Theme: Sleep disorders. 4. 2. 199-The following maternal conditions are known to cause adverse effects on the neonate: 1. Btk is necessary for transducing signals initiated by ligation of the pre-Breceptor. The defect is a deficiency in the wiskott -aldrich protein – which play a role in cytoskeletal organization and possibly intracellular signaling. Hyperthyroidism. eczema and immunodeficiency. Sleep walking occurs during REM sleep. Alternative of the parents responses to the children’s night time behaviour so as to prevent reinforcement of the problem. Night terrors: child wakes up. Occur in up to 3% of toddlers. The child then starts to breath in half a minute or s o. Breath holding attacks should be investigated with CT scan. Microvilli on T -cell appear abnormal. Period of disturbed behaviour may last up to 15 minutes. 5. Sleepwalking: Occur in a state of deep. Teaching Notes for Question 199 Theme: Maternal conditions causing adverse effects on the neonate. Reinforced when parents show concern. the child goes blue in the face especially around the face. This gene encodes a protein tyrosine kinase – B-cell tyrosine kinase (Btk). Night terrors occur in the stages of deep sleep. Occasionally. Toxoplasmosis. may be difficult to comfort. Can be used by children to express anger towards parents. 2. Precipitated by minor upsets. SLE. Crdinal features are thrombocytopenia. Sleep disorders are never treated with drugs.

3. Alkalosis will have the opposite effect. 4. the neonate may present with thyrotoxicosis due to the transplacental passage of TSI. In anaemia.3-DPG levels. Toxoplasmosis leads to congenital infection by transplacental t ransmission. High altitude.Temperature – A rise in temperature shifts the curve to the right i. The ewborn presents with the classic triad of hydrocephalus. Alkalosis. This curve is affected by a number of factors: 1.e a higher PO2 is required for haemoglobin to bind oxygen. A rise in 2. 2. Increased red cell volume and MCH. Increased 2-3 DPG.PEDIATRICS EXAMINATION REVIEW Maternal SLE is a risk factor for neonatal lupus syndrome. An increase in CO2 (as is found in peripheral tissues) will cause a fall in PH. Teaching Notes for Question 200 Theme: Oxygen uptake. These symptoms are frequentely short -lived i. Raised temperature. the MCHC is reduced. rather than the MCH or MCV. The skin is frequently involved with malar rashes and there can be haematological and cardiac abnormalities. Splenomegaly can restrict intrauterine growth and lead to premature de livery.3 -DPG shifts the oxygen dissociation curve to the right releasing more O2. chorioretinitis. The most frequent heart abnormality is congenital heart block – 90% of mothers whose infants have congenital heart block are anti Ro(SSA)Ab positive. The greater affinity of foetal haemoglobin for oxygen as compared to its adult counterpart is a result of the decreased binding of 2. 3. The oxygen haemoglobin dissociation curve relates the saturation of haem with oxygen as a % (the y axis) to the PO2 in mmHg(on the x axis).e as long as the circulating antibodies persist in the baby’s circulation. Haem is a complex of prophyrin and ferrous iron. with an increase in the availability of oxygen to th e tissues. and the associated decrease in affinity of haemoglobin for oxygen contributes to the transfer of oxygen from the blood in to the tissues. 4. This is known as the Bohr ef fect. Hae moglbin is composed of four globin subunits (in normal adults – two alpha and ywo beta globin chains) each of which contains a haem group.60% of third trimester infections and 20% -30% during the first two trimesters. 121 . 99% of the oxygen which dissolves in blood is transported by the oxygen carrying compound haemoglobin.High altitude results in a rise in 2.this substance is formed by glycolosis via the Embden Meyerhof pathway.2. The rate of transmission is +/ . and the curve is right shifted. If hyperthyroidism is due to Grave’s disease or Hashimoto thyroiditis. increasing the release of oxygen to the tissues.3 -DPG by the foetal gamma globin chain.3 -DPG concentrations in rbcs. it is the MCHC which affects the oxygen dissociation curve. Pathology can be secondary to placental leukaemic infiltrates. It binds to the beta -globin chains of deoxyhaemoglobin and causes oxygen to dissociate from Hb.With regards rbc indices. 5. The neonate presents with clinical features of SLE due to transplacental passage of maternal Ab. 2. 5. and cerebral calcification.e oxygen uptake by haemoglobin is decreased.PH – a fall in PH shifts the curve to the right i. Maternal chronic myeloid l eukaemia can have adverse effect on pregnancy – fetal/neonatal mortality is 16 -38%. anaemia and infectious complications. 200-en uptake by haemoglobin is characterized by: 1.

PEDIATRICS EXAMINATION REVIEW Questions (PART 2) SELECT ONLY ONE ANSWER 122 .

Hyperbaric oxygen B. Anti-smooth muscle antibodies in Primary biliary cirrhosis C. Goblet cell D. vomiting. Anti-liver/kidney microsomal antibodies (anti LKM-1) in type I autoimmune hepatitis B. Endothelial cell C. Factor V Leiden D. CT brain and lumbar puncture D. The carboxyhaemoglobin (COHb) level is 25%. Type II pneumocyte 5-Which of the following conditions with renal involvement is the most likely to have normal complement levels: Options: 123 .A 14-year-old boy is rescued from a burning building and is brought to the hospital. Anti liver cytosol antibodies in primary sclerosing cholangitis 4-hich one of the following cells in the lung parenchyma produces surfactant? Options: A. Which of the following is the best treatment for this patient? Options: A. Type I pneumocyte E. High flow oxygen via a facial mask C. Chicken pox E.PEDIATRICS EXAMINATION REVIEW 1. diarrhoea and abdominal pain. Alveolar macrophage B. Anti-nuclear antibodies in Primary biliary cirrhosis D. Meningococcal septicaemia 3-Choose the correct matching pair of disease and antibody out of the following options:Options: A. Intravenous fluid and oxygen via nasal prongs E. Warfarin therapy B. Neonatal purpura fulminans C. He is confused. He has nausea. Urgent senior surgical opinion to exclude bowel obstruction 2-Low protein C levels are not seen with: Options: A. Anti-mitochondrial antibodies in type II autoimmune hepatitis E.

High -dose steroids 124 . Type II cryoglobulinaemia E. Interferon-1-Beta C. 7-The best treatment option for a patient with Usual Interstitial Pneumonia (UIP or CFA (Cryptogenic fibrosisng alveolitis) is : Options: A. Subacute bacterial endocarditis D. Grade IV lupus nephritis B. Lung transplantation B. E. Visceral leishmaniasis is not found in the southern European countries.PEDIATRICS EXAMINATION REVIEW A. Azathioprine D. Yellow fever is endemic in India and South -East Asia. Cyclophophamide E. Malaria is only endemic in the tropics. D. The distribution of infection wi th Schistosoma mansoni and Schistosoma haematobium are similar. IgA nephropathy (Buerger’s disease) 6-Which of the following statements about the epidemiology of tropical diseases is correct? Options: A. Post-streptococcal glomerulonephritis C. B. C. Chaga's disease is confined to South America.

Adenosine deaminase deficiency D. LBO (Large bowel obstruction) B. Quinidine-induced thrombocytopenia 125 . Toxic Megacolon D. Haemolysis following transfusion of blood bearing mismatched blood group antigens C. Ankylosing Spondylitis 9-A 6 month old boy with severe eczema presents with easy bru ising and two nose bleeds. Chagas Disease E. He has had 3 previous admissions to hospital with pneumonia and has required two courses of antibiotics for otitis media. Ataxia telangiectasia C. SBO (Small bowel obstruction) C. Primary antibody deficiency B.PEDIATRICS EXAMINATION REVIEW 8-What is the diagnosis that can you infer from the X-ray abdomen? Options: A. Wiskott Aldrich syndrome 10-All of the following are type II hypersensitivities except: Options: A. The Arthus reaction D. C4 deficiency E. He is below the 2nd centile for weight. What is the most likely underlying diagnosis :Options: A. Production of autoantibodies to acetylcholine receptors in myasthenia gravis B. Hyperacute graft rejection E.

E. Ataxia telangiectasia E. Documented seroconversion confirms infection. Risk of fetal infection is increased with increasing gestational age. Always clinically apparent at birth D. Characterised histologically by tubule dysplasia and cystic tissue throughout the renal parenchyma C. C. Kown to exist in 4 genetic forms 12-Which of the following statements regarding toxoplasmosis in pregnancy is false? Options A. 13-A 9-year-old has a cranial CT scan. Which of the following diagnoses is l east likely to be associated with the radiologist’s report? Options: A. Maternal toxoplasma-specific IgM may persist for over a year.PEDIATRICS EXAMINATION REVIEW 11-Polycystic kidney disease is: Options A. Sturge-Weber syndrome C. B. The radiologist reports the presence of intracranial calcification. bone and anterior pituitary 126 . Treatment with pyrimethamine and sulphadiazine is recommended during the first trimester to reduce risk of foetal infection. It is associated with pulmonary hypoplasia C. Hypoparathyroidism 14-Choose the one correct statement regarding a Congenital diaphragmatic hernia:Options A. Characterised histologically by micro . Foetal abnormality may occur in cases of maternal infection occurring 6 months pre-conception. It usually presents with vomiting 15-Regarding Histiocytosis X :Options: A. It is always symptomatic at birth E. Toxoplasmosis D.and macroscopic cysts distributed throughout the renal parenchyma E. Subdural haematoma B. It is most commonly seen on the right side B. D. Usually fatal at term B. It is associated with oligohydramnios D. It is a cystic disease of lung.

Ageing is associated with altered insulin signalling. Ageing can be slowed in animal models by free radical scavengers. The data are positively skewed B. Down's Syndrome D. Possible diagnoses are: Options: A. D. E. The median and interquartile range is a good way of summarising the data 18-Which of the following hypotheses on the mechanisms of ageing is least accurate? Options: A. Noonan's syndrome B. Turner's syndrome E. Ageing is thought to be due to shortening of the telomeres. It is a rare cause of pneumothorax 16-A girl with an unusual facial appearance is found to have coarctation. 127 . most are men 20 -40 years old D. B. CXR shows small lungs with prominent markings and honeycombing C.PEDIATRICS EXAMINATION REVIEW B. 80% of patients are smokers. The median is greater than the mode E. C. Ageing can be reduced in mammals by caloric restriction. Edward's syndrome 17-A study measures the heights of 13 -yr-old children. The data are normally distributed C. Ageing is caused by nitric oxide free radical-induced mitochondrial damage. William's syndrome C. Most important treatment strategy is steroids E. Which is the best conclusion to draw from the data? Options: A. The mean is the best measure of central tendency D.

Right lower lobe consolidation 20-Which one of the following is true regarding cancer genetics?: Options: A. Left lower lobe consolidation B. Membranoproliferative glomerulonephritis B. Left lingular collapse E. B. A proto -oncogene is an abnormal gene that encodes a protein involved in regulation of cell growth or proliferation and that can be mutated into a cancer -promoting gene. A gene whose product is involved either in transforming cells in culture or in inducing cancer in animals is called a proto-oncogene.PEDIATRICS EXAMINATION REVIEW 19-What is the diagnosis? Options: A. Left lingular consolidation D. D. and BRCA1 are oncogenes C. Any gene whose encoded protein directly or indirectly inhibits progression through the cell cycle and in which a loss-of-function mutation is oncogenic is an oncogene. Shunt nephritis D. Left lower lobe collapse C. A proto-oncogene is transformed onto an oncogene by changing the protein -coding segment or by altering its expression. E. Goodpasture’s disease 128 . RB. APC. 21-Which of the following renal disorders is associated with normal serum complement levels? Options: A. Post-infectious glomerulonephritis E. All forms of lupus nephritis C.

Primary hyperaldosteronism C. Causes the release of CRP. it follows a stereotypical cascade. Persistent vomiting D. the half -life of which is several days C. Is always harmful E. a potent chemokine B.0 mmol/l Urine chloride 10 mmol/l Options: A.PEDIATRICS EXAMINATION REVIEW 22-What is the most likely explanation for these biochemical changes in a 15 -year-old girl with erosive changes in her mouth? Na 138 mmol/l K 3. Primary hyperparathyroidism B. Anorexia nervosa E. Ehlers Danlos syndrome 23-A patient presents with acute infective arthritis. Causes the release of C8a. Sinus tachycardia D. Atrial flutter B. May be defined as increased vascular permeability accompanied by an infiltration of inflammatory cells D.4 mmol/l P04 1. Ectopic atrial tachycardia 129 . AV reentrant tachycardia due to accessory pathway E. Supraventricular tachycardia C. which is not regulated 24-What does this ECG of an 8-year-old boy with Ebstein’s anomaly show? Options: A.2 mmol/l HCO3 36 mmol/l Ca 2. The resulting inflammation: Options: A. Once initiated.

Choose the correct statement regarding alpha 1 -antitrypsin (AAT): Options: A. It is not seen in babies who haven't been started on feeds yet D. It is commonly associated with gas in the biliary tree C. Prolonged vomiting is an indication for high -dose oral magnesium supplementation E. Hungry bone syndrome is associated with reduced serum magnesium 27-All these are major clinical features of neurofibromatosis -1 (NF-1) except: Options A. Severe exfoliative psoriasis E. Café au lait patches 28-Choose the one correct statement regarding necrotising enterocolitis: Options: A. Axillary freckling B. Tibial pseudoarthrosis C. It can be prevented by the use of oral antibiotics 29. Cortical tubers D. Lisch nodules E. the following statement is not true: Options: A. Neuromuscular symptoms are similar to those in hypocalcaemia D. High-dose aspirin therapy 26-In a patient with hypomagnesaemia. Lesch-Nyhan syndrome B.PEDIATRICS EXAMINATION REVIEW 25-Which of the following conditions is not associated with hyperuricaemia : Options: A. Polycythaemia rubra vera D. Thiazide diuretic therapy C. Hypocalcaemia may occur C. Recent cisplatin treatment could have been the cause B. It may lead to intestinal obstruction a few weeks later E. It is a membrane bound tyrosine kinase 130 . It usually involves the lower colon B.

Night time insomnia can be a feature D. Cystic fibrosis D. Global areflexia is a recognised association E. AST 34 Ca2+ 1. It is associated with cataplexy B.6 . ALP 320 MCV 108 Bili 12. Both her sisters (age 14 and 19) are greater then 170cm tall and are well. Ulcerative colitis 32-Which of the following is not true of narcolepsy? Options: A. It exhibits X-linked inheritance pattern C. It may be associated with hypnopompic hallucinations 131 . Addison’s disease E. She has recently started developing breasts. The affected pupil is always larger than the unaffected B. There is denervation supersensitivity of the pupil D. Both pupils may be affected C.9 U&E normal. Crohn’s disease B.5 .7 . PO4 0. It is associated with early -onset REM sleep E.PEDIATRICS EXAMINATION REVIEW B. WCC 7. Hb 10. Deficiency presents as cirrhosis in children D. TFTs normal What is the underlying diagnosis ? Options: A. Over 75% of cases of emphysema in the UK are associated with the deficiency of this enzyme C. She is 145 cm tall and weighs 48 kg. The low levels of the enzyme in deficiency syndromes are due to lack of synthesis of the variant enzyme E. Ptosis is rare 31-A 17 year old girl presents with primary amenorrhoea. Emphysema in deficiency syndromes is characterised histologically by inclusion bodies 30-Which of the following is not a feature of the Holmes –Adie syndrome? Options A. Coeliac disease C.

Rheumatoid arthritis E. The technique can be performed on uncultured cells B. Production of memory plasma cells D. EEG will help in the diagnosis C. A quantitatively increased antibody production as compared to the primary immune r esponse E. Isotype switching to IgG B. An aura and postdrome stage is present as in adul t patients E. The technique is used instead of light microscopy for routine chromosome analysis E. Requirement of help from T-cells 132 . Uraemia 36-The secondary immunoglobulin response is not c haracterized by:Options: A. Other members of the family may be affected D. Rheumatic fever C. The technique can detect small deletions in DNA D. Sumatriptan is not licensed for use in children 34-The following statements about techniques using FISH (fluorescent in situ hybridisation) are true except: Options: A. Pneumococcal pneumonia D. Tuberculosis B. The technique requires specialised microscopes C.PEDIATRICS EXAMINATION REVIEW 33-The following are all true about migraine in children except: Options: A. More than 50% of headaches are unilateral B. The technique can be used to map the position of genes on a chr omosome 35-Cardiac tamponade may occur with all of the following except: Options: A. Production of immunoglobulin with higher affinity to the antigen C.

neck and hands. Myelomatous deposit E. Complement levels 133 . Osteopetrosis 38-In the investigation of a patient who is admitted following 3 dizzy spells each markedly improved by eating:Options: A. Serum C -peptide will not be raised in exogenous insulin use E. c-reactive protein and erythrocyte sedimentation rate C. Serum insulin. Subperiosteal resorption of HPTH (Hyperparathyroidism) B. AVN (Avascular necrosis) talus D. glucose and C-peptide should be taken every morning for 7 days D. What are the most appropriate tests to request? Options: A. C. joint aches and a red rash on her face. Full blood count and chemistry D. Urine sulphonylurea levels are unhelpful B. HPOA (Hypertrophic Osteoarthropathy) C. A family history of scars in the neck is irrelevant 39-A 16-year-old girl presents with fever. Anti-nuclear antibody and erythrocyte sedimentation rate E. Anti nuclear antibody and c-reactive protein B. She attributed the rash to sev ere sunburn and says that she has sensitive skin.PEDIATRICS EXAMINATION REVIEW 37-What is the abnormality seen in this girl with bone pain? Options: A. with the doctor called when the blood sugar reaches 4mmol/l. A 72hr fast should be organised.

Levels are elevated in >90% of cases of acromegaly E. Serum creatinine C. Can crawl C. E. Gold salts may lead to nephrotic syndrome. Elevated levels produce gynaecomastia without galactorrhoea B. Can stand alone D. What is the most useful investigation? Options: A. Low dose methotrexate may be beneficial 134 . Has lost the Moro reflex 42-An 18-year-old man is referred to the outpatient clinic as his general practitioner has found him to have microscopic haematuria. Iridocyclitis usually res ponds to topical steroids. Metoclopramide increases prolactin production D. Renal biopsy 43-The following statement is false regarding treatment of Juvenile Idiopathic Arthritis (JIA):Options A. H e also has bilateral sensorineural deafness with a sister and younger brother who are similarly afflicted. Audiometry E. Urine microscopy B. By the age of 9 months a child: Options A. Can play peek -a-boo E. Can sit with support B. B.PEDIATRICS EXAMINATION REVIEW 40-Which of the following is true about prolactin? Options A. Anti-GBM antibodies D. Levels are increased by cimetidine 41-Choose the incorrect statement. Steroids are the agents of first choice. C. D. Hydroxychloroquine is associated with ocular toxicity. Damage to the pituitary stalk reduces prolactin levels C.

Consolidation right lower lobe B. Collapse right lower lobe C. Collapse lingular lobe D. Perforation E. Bilateral pneumothoraces 135 .PEDIATRICS EXAMINATION REVIEW 44-What is the abnormality shown on Chest X -ray? Options: A.

Often leads to chronic haemolysis. C. C5-C9 deficiency and mycoplasma infection B. C3 def is associated with partial lipodystrophy E. C1q deficiency and angio -oedema C.PEDIATRICS EXAMINATION REVIEW 45-What is the diagnosis? Options: A. Background retinopathy 46-All of the following statements about Glu cose-6-phosphate dehydrogenase deficiency are false except: Options: A. Endocarditis D. Is caused by mutations of the G6PD gene on chromosome 4. CMV retinitis E. D. C2 deficiency and rheumatoid arthritis D. Is more common in women than in men. 47-Which of the following statements correctly describes the clinical phenotypes which are associated with a deficiency of the various complement compon ents? Options: A. B. A raised C4 and low C3 in SLE is associated with acti ve disease 136 . Proliferative DM retinopathy B. E. Requires treatment with desferrioxam ine to prevent iron overload. Grade 3 hypertensive retinopathy C. Is associated with haemolytic crises induced by sulfonamides.

Soft aortic component of second heart sound D. capillary refill time is 5 seconds and he has a purpuri c rash over his lower limbs. He is not responsive to pain. Anti-phospholipid antibodies in Primary biliary cirrhosis C. Give immediate IM ceftriaxone before securing IV access E. Check a capillary blood gas 51-A patient is noted to have a small left pupil. A colleague has been trying for 5 minutes to secure intravenous access when you arrive. Anti-mitochondrial antibodies in Primary biliary cirrhosis D. He has been unwell for 1 2 hours with fever and has vomited once. The next step should be: Options: A. Oculomotor nerve B. Systolic thrill E. Continue to try for IV access and then give 20mL/kg fluid if success ful B. His heart rate is 160/min. Length of murmur in systole 50-A two-year-old boy is brought to the resuscitation room. Facial nerve D. Anti-liver cytosol antibodies in primary sclerosing cholangitis 49-All of the following are clinical features of severe aortic stenosis exce pt: Options: A. Third heart sound C. Vagus nerve E. On examination. Trigeminal nerve C. Anti-liver/kidney microsomal antibodies (anti LKM-1) in type I autoimmune hepatitis B. There is also a ptosis p resent in this eye. Check blood sugar C. Where is the lesion likely to be? Options A. ANCA in type II autoimmune hepatitis E. Fourth heart sound B. Secure intraosseus access and give 20mL/kg fluid D. his airway is patent and he is breathing oxygen via a mask with non -rebreathing bag with oxygen saturations of 100%.PEDIATRICS EXAMINATION REVIEW 48-The following autoantibodies are commonly found in the named disease: Options A. His mother has noted that the left side of his face is dryer than normal. Cervical sympathetic trunk 137 .

Those with diabetes mellitus E. The trial would have been better run against placebo 55-Which of the following is correct regarding atrial flutter? Options: A. Those with coeliac disease 54-The lipid-lowering effect of a new statin is compared to atorvastatin. Ptosis C. Immunosupressed people due to disease or treatment B. Pupillary constriction B. What is the best conclusion to draw? Options A. Which of the following is NOT a symptom of Horner’s syndrome? Options: A.PEDIATRICS EXAMINATION REVIEW 52-A patient is diagnosed as having a Horner's syndrome following carotid surgery. Those with chronic heart disease C. Critical level is set at 0.01 and difference is found (p = 0. A larger sample should have been used B. Can be terminated with intravenous adenosine E. Those with chronic renal failure D.001). Usually causes a narrow complex tachycardia at 150 bpm C. Absence of sweating on face and neck E. Is often associated with an irregular pulse D. The p-value associated with this statin means that it is a clinic ally significant new drug E. Is usually left atrial in origin B. The probability of a type I error occuring is 1% D. Lack of lacrimation 53-Which of the following sub-group of populations is the influenza vaccine not recommended for?:Options A. Sinking in of one eye D. Implies the presence an accessory pathway 138 . The alternative hypothesis should be accepted C.

PEDIATRICS EXAMINATION REVIEW 56-The following statements are true about Tetralogy of Fallot (TOF) except: Options : A. Left tension pneumothorax E. A renal consultation is advised if the serum creatinine rises above 250 µmol/l. The target HbA1c is about 8%. This disorder results in a left to rig ht shunt 57-In terms of the NICE guideline of screening for diabetic nephropathy in Type 2 patients: Options: A. 58-What can you infer from the Chest X-ray? Options: A. Left pneumothorax B. D. should be on an ACE inhibitor. Calculating ratios of albumin and creatinine in the urine is not as useful as the total urine protein level. Left hilar adenopathy 139 . Left lower lobe collapse C. Children with TOF are susceptible to cerebral abscess B. Blood pressure is less important than reducing the degree of proteinuria. if no contraindications. TOF is associated with dextroposition of the aorta E. B. The second heart sound is soft and single D. The chest X-ray shows oligaemic lung fields C. Left lingula collapse D. E. All patients with confirmed microalbuminuria. C.

E. What is the likely result? Options: A. Thymus C. Pancreas 60-Following a dislocated shoulder in a rugby match that gets out of hand. 5–10% of cases manifest with meconium ileus at birth B. a 10-year-old boy presents with a chronic cough. The patient may lose the ability to adduct the arm. In 70% of patients. Pancreatic abnormalities are present in 80% of patients D. D. The teres major atrophies. Bone marrow D. C.PEDIATRICS EXAMINATION REVIEW 59-Which of the organs below is considered an immunologically privileged site : Options: A. and she has noticed that his sweat tastes excessively salty. Testicle E. The patient may have a wrist drop. the genetic abnormality is a four -base pair deletion at the locus on chromosome 7 C. Staphylococcus aureus and pseudomonas aeruginosa are two pathogens most commonly isolated from the sputum of patients with this condition 140 . recurrent lung infections and clinical evidence of obstructive pulmo nary disease. A loss of sensation may occur in the lateral forearm. B. All the above are features of this condition EXCEPT: Options: A. Skin B. The rounded contour of the shoulder disappears. 61-Steve. The primary defect is in the transport of chlor ide ions across epithelial tissue E. a patient’s axillary nerve is damaged. His mother reveals that when he was 2 years old he was diagnosed as having ‘malabsorption’.

blood 2+ proteins. She has been previously fit and well except for a history of mild intermittent arthralgia affecting both hands. E. Syphilis E. C. She smokes 20 cigarettes per day and drinks 15 units of alcohol per week. He recently visited his GP for a deep IM steroid injection. Posterior cord of the brachial plexus. * On examination she has no focal neurology. * Urinalysis tests 3+.8 mmol 11 seconds 39 seconds Positive 1:320 Negative Not detected What is the likely diagnosis? Options: A. SLE B. Systemic sclerosis 63-An elderly man presents to casualty with a wrist drop and pain radiating down his right arm. 141 . The long thoracic nerve. The medial cord of the brachial plexus.9 g/dl 83 fl 3.6C. Her blood test results reveal: Hb MCV WCC Platelets CRP ESR Sodium Potassium Urea Creatinine Albumin Total protein LFTs BM Clotting screen: PTT APTT VDRL Drug screen of urine Plasma alcohol 9. B. * Fundoscopy reveals silver wiring and AV nipping bilaterally. D. Epilepsy C. Her only medication is the OCP. * BP 160/98 mmHg.PEDIATRICS EXAMINATION REVIEW 62-A 26-year-old airhostess is brought to the A&E depar tment following a witnessed grand mal seizure.1 mmol/l 178 mmol/l 30 g/l 80 g/l Normal 7. The ulnar nerve. The C5 spinal root. The probable site of the injection was the: Options: A.1 x 109/l 117 x 109/l 2 g/l 71 mm in the first hour 134 mmol/l 5. * There is slight puffiness of her fingers and she has low -grade pyrexia of 37. Pulmonary embolism D.1 mmol/l 10.

Rheumatic fever 65-Milkmaid grasp is characteristically seen in which of the following conditions? Options A. He should not have any further immunisations B. Lyme disease E. Scarlet fever D. which his mother says has been present for one week and has increased in size. On examination. Hemiballismus B. Typhoid B. The presence of jaundice would make which one of the following diagnoses more likely? Options: A. Torticollis E. Malaria C. Toxocariasis D. arthralgia and headache and a red. Gilles de la Tourette syndrome 66-A 2-year old boy is admitted wit h fever. Huntington’s disease D. He should have all immunisations except pertussis C. Lyme disease E. Cat scratch fever 67-A two-month-old child had his first DTP injection 3 days previously and his mother has returned because his thigh is swollen. He should receive the whole cell pertussis vaccine (as DTP) as usual at his next immunisation 142 . What will you recommend for this child in the future? Options A.PEDIATRICS EXAMINATION REVIEW 64-A boy presents with fever. Which is the most likely diagnosis? Options: A. most of the anterolateral surface of the thigh is red and indurated. Kawasaki disease B. Infectious mononucleosis C. He should receive the oral pertussis vaccine when next immunised E. Sydenham’s chorea C. annular rash in his axilla. He should receive the acellular pertussis vaccine when next immunised D.

Ethylene glycol C. The following blood test results are found. K 5. MDMA 71-The most important cause of death in infectious mononucleosis is: Options: A. Chronic hepatitis B infection (high infectivity) E. What has he taken? Options: A. Cl 110. Pco2 4. HCO3 15.5. Acute hepatitis B infection B. D. Glue E. Urine osmolality is >500 mosm/kg E. E. B. Na 135. Chronic hepatitis B infection (low infectivity) D. C. Past hepatitis B infection C.29 . Heart failure. Respiratory failure. He is unconscious and he has a rash around his mouth.PEDIATRICS EXAMINATION REVIEW 68-You see a 15-year-old girl in clinic with the following results: * Hepatitis B surface antigen: positive * Antibodies to hepatitis B surface antigen: negative * Hepatitis B 'e' antigen: negative * Antibodies to hepatitis B core antigen: IgG positive * Antibodies to hepatitis B 'e' antigen: positive Which of the following statements best describes her clinical condition? Options: A. Oedema may occur due to fluid overload D. Methanol B. Kidney failure. Splenic rupture. pH 7. Plasma urate levels are elevated C. Opiates D. Previously vaccinated against hepatitis B 69-In the syndrome of inappropriate ADH secretion (SIADH): Options: A. Liver failure. Serum globulins are high 70-A 14 year-old boy is seen in the Ambulance and Emergency Department. Plasma sodium is usually normal B. 143 .

Ulnar nerve 144 . C5 spinal root E. T1 spinal root D. Can produce hyponatraemia due to Diabetes Insipidus C. Optic neuritis B. Which of the following diagnoses best fits his sympt oms? Options: A. Medial cord of the brachial plexus C. right arm and shoulder during a game of tennis. Retinitis pigmentosa 74-A middle aged lady presents to Casualty with a wrist drop and pain radiating down her right arm after sustaining an injury to her neck. On examination. Are a feature of porphyria cutanea tarda D. Anterior uveitis C. “Non -organic” symptoms E. Can be precipitated by paracetamol B. Posterior cord of the brachial plexus B. he reports having only central vision over an area of approximately 1 m by 1 m. Are precipitated by the contraceptive pill E. Are associated with malabsorption 73-A 17-yr-old male medical stu dent presents with a sudden onset of tunnel vision. He reports this at a distance of 3 m and also at 6 m.PEDIATRICS EXAMINATION REVIEW 72-Acute attacks of porphyria: Options: A. The probable site of the injury was the: Options: A. Methanol poisoning D.

Osteoporosis B. Her only medication is the OCP. Measuring ACTH levels E.PEDIATRICS EXAMINATION REVIEW 75-A 26-year-old air hostess is brought to A&E following a witnessed grand mal seizure. tachycardia. Septic arthritis D.1 mmol/l 10. On examination she has no focal neurology.1 mmol/l 178 mmol/l 30 g/l 80 g/l normal 7. but at an OPD appointment 3 months later is complaining of worsening right hip pain poorly res ponsive to NSAIDs. She responds well to prednisolone and azathioprine. Trochanteric bursitis 76-A 35-year-old woman who complained of easy bruising. Low-dose dexamethasone suppression test B. What is the most likely cause of her hip pain? Options: A. Avascular necrosis C. O2 sats 94% on room air. CRH stimulation test 145 . There is slight puffiness of the fingers and low -grade pyrexia of 37. weight gain and striae is being worked up for Cushing’s syndrome.8 mmol The patient then develops acute dyspnoea and examination reveals decreased air entry at the right base. Measuring cortisol levels D.1 X 109/l 117 X 109/l 2 g/l 71 mm in the first hour 134 mmol/l 5. High-dose dexamethasone suppression test C. X-ray is normal but bone scan shows increased uptake in the hip. Which is the next investigation to confirm the diagnosis? Options: A. Fundoscopy reveals silver wiring and AV nipping b ilaterally. 2+ protein. BP 160/98 mmHg.6°C.9 g/dl 83 fl 3. Results: Hb MCV WCC platelets CRP ESR Sodium potassium urea creatinine Albumin total protein LFTs BM Clotting screen: PTT APTT VDRL Drug screen of urine Plasma alcohol 11 seconds 39 seconds positive 1:320 negative not detected 9. Stress fracture E. Urine analysis tests show 3+ blood. BP 105/55. she smokes 20/day and drinks 15 units of alcohol per week. She has been previously fit and well except for a history of mild intermittent arthralgia affecting both hands. Her 24 -hour urinary free cortisol is 450 nmol.

Preexisting immunocompromise is a risk factor C. CXR shows hazy shadowing in the right mid and lower zones. On examination the child is afebrile and dehydrated with a depressed anterior fontanelle. He has been vomiting non-billious breast milk for the last 3 weeks. Rifampicin D. Th e operation is uneventful however she is admitted to the Intensive Therapy unit for 48 hours postoperatively. however 10 days after admission she develops a fever. Penicillin C. headaches and a non -productive cough. Strict barrier nursing is essential to contain the infectious agent B. Ciprofloxacin E. Cryptococcus neoformans 78-A 6 week old male baby presents with a 2 day history of multiple seizures. Therapy is started and 5 days later he becomes acutely jaundiced with red discolouration of the urine. Pontiac fever is a rarer and milder form of the disease E. Streptococcus pneumoniae B. The intravenous (iv) fluid of choice for administration would be: Options: A. Investigations show hyponatraemia and mildly deranged LFTs. Examination reveals no focal signs of infection. iv Hypertonic saline 79-Regarding Legionnaire's disease:Options: A. Cytomegalovirus E. Options: A. Tetracycline 146 . Klebsiella pneumoniae C. Which of the following drugs is the likely cause :Options: A.PEDIATRICS EXAMINATION REVIEW 77-A 26-year-old woman is admitted to hospital for cadaveric renal transplantation. iv sodium bicarbonate E. however after 48 hours the fevers continue and a repeat CXR shows left lower lobe consolidation. Rifampicin alone is as effective as erythromycin for the treatment D. Cefotaxime B. She is treated with cefotaxime. iv 5% dextrose D. Blood cultures are taken and a CXR is unremarkable. diarrhoea. iv isolyte – P C. Diagnosis can be made from stool culture 80-A 15 year old boy is admitted with pneumonia. She is given treatment with prednisolone and cyclosporin. Pneumocystis carinii D. iv 50% calcium gluconate B. Ultrasound of the abdomen shows hypertrophic pyloric stenosis.

Thick and thin blood film B. B. E. Cyclosporin A. during a trip to an art gallery. Most children with Down's syndrome are born to women over the age of 35 years C. Congenital adrenal hyperplasia. Cushing's syndrome. 83-A family attends genetic counselling.PEDIATRICS EXAMINATION REVIEW 81-Seven weeks after returning from rural Ghana. Anterior iscaemic optic neuropathy D. Optic neuritis C. Amaurosis Fugax E. HIV antibody test 82-A 15-year-old is undergoing investigation for hirsuitism. Which of the following statements is most accurate? Options: A. Phenytoin. Blood cultures E. Chest radiograph D. Moxonidine. A karyotype of 46XX excludes a diagnosis of Down’s syndrome E. The pupil appears dilated and does not constrict to light. What is the most likely diagnosis : Options: A. C. Amniocentesis should only be offered to confirm the diagnosis of Down’s syndrome if the family will consider termination of the pregnancy D. She complains of discomfort in the eye and thinks that difficulty with perception of colour was the first problem that she noticed. visual acuity on the left is down to light perception. Central retinal artery occlusion 147 . Which of the following would not be implicated in the aetiology? Options: A. CMV retinitis B. The least usefu l test is: Options: A. D. a 16 -year-old boy develops fever and rigors. but there is no need to karyotye the affected child B. although does when a torch is shone in the right eye. They have one child affected with Down’s syndrom e and are considering another pregnancy. Both parents should have karyotype analysis. On examination. A nuchal scan together with bHCG and PAP (pregnancy associated protein) between 11 and 13 weeks of pregnancy gives an accurate risk of Down’s syndrome in >80% of cases 84-A 17 year old student develops a deterioration in vision in her left eye over three day s. IgM for viral haemorrhagic fever C.

He is lucid with a GCS of 15.6 g/l RBC 2 /ml WBC 21/ml (100% lymphocytes) CSF glucose 4.5 mmol/l CRP 39g/l ESR 56 mm in the first hour RF. His blood test results show: Hb 13. ENA and ANCA negative CSF analysis: opening pressure 14cmH2O protein 0. and rash. Adult onset Still's disease C.9 X 109/l plasma glucose 6. He is then hospitalised with severe headache. Aminoglycosides C. Prednisolone B. Pyridostigmine D.8g/dl plats 210 X 109/l WCC 4. Behcet’s syndrome D. ANA. pleuritic pain.PEDIATRICS EXAMINATION REVIEW 85-A 29 year old Cypriot man gives a history of recurrent pain and swelling of the left knee. Lithium E. Familial Hibernian fever E.1 mmol/l no organisms seen on microscopy 48 hour culture of CSF negative What is the diagnosis? Options: A. photophob ia and neck stiffness. Familial Mediterranean fever B. Non Hodgkin’s lymphoma 86-Which of the following do not tend to make the symptoms of myasthenia gravis worse? Options: A. He also describes recurrent episodes of fever. Quinidine 148 .

which muscle would you look for w asting? Options: A. When penicillamine is administrated then urinary copper level rise C. Is inherited as an autosomial dominant trait D. She develops a painful hand at night. What is the diagnosis? Options: A. Fallot’s tetralogy C. Most common presentation is with a manic depressive illness 149 . Flexor digiti minimi E. Abductor pollicis brevis C. which of the following statements is true : Options: A. Extensor pollicis 89-In a patient that has been previously diagnosed with Wilson’s disease. Eisenmenger’s syndrome E. Lateral two interossei B. Serum cerruloplasmin levels are raised B. Transposition of great arteries D. In view of the probable aetiology. Pulmonary thromboembolic disease B.PEDIATRICS EXAMINATION REVIEW 87-This 15-year-old was cyanosed on exertion. May result in retinal abnormalities E. Mitral stenosis 88-A patient has hypothyroidism. Medial two lumbricals D.

5)(q35. There is a translocation between the short arm of chromosome 2 and the short arm of chromosome 5 D. The patient may be jaundiced during the immune phase D. drinking 3 -4 bottles of water through the night. Her parents tell you that she has always been a very thirsty child. Corneal opacities. It is usually transmitted by rat bite B. He is likely to be infertile E. A. He has more than 46 chromosomes B. On immunofluorescent studies. Cataract. Minimal change disease is a histological diagnosis 91-A child has had his chromosomes analysed in the course of various investigations and the result is: 46 XY. Albumin is routinely given to children with proteinuria D. Long-term cyclophosphamide therapy is beneficial in the steroid resistant group E. The result shows all his genetic defects C. Metabolic acidosis with a wide anion gap. Nephrocalcinosis. urine dipstick shows 1+ glucose. Which of the following features are you likkely to find on examination and investigation. and 1+protein. B. The organism may be isolated from urine during the septic phase E.3}. Hypervolaemia is a common problem C. It is a notifiable disease C.p21.PEDIATRICS EXAMINATION REVIEW 90-Which of the following statement(s) regarding childhood minimal -change nephrotic syndrome is/are true?: Options. 150 . E. Options: A. C. deposition is seen along the glomerular baseme nt membrane B. t (2. Hyperkalaemia. Ciprofloxacin is the treatment of choice for severe forms 93-A 9 months old baby girl is brought to you with failure to thrive and rickets. There is an increased risk of him having a child with difficulties 92-In leptospirosis which one of the following is true? Options: A. D. and having soaking wet napping. Which of the following statements is correct?: Options: A.

Do a lymph node biopsy to exclude concomitant lymphoma. Her partner has a microcytic anaemia. Referre him for a bone marrow transplantation. Start IV fluids & allopurinol. Deep venous thrombosis. but there are small blasts in the blood film. his full blood count is normal . Which diagnosis is impossible in the baby? Options: A. 96-The mother of a baby in the postnatal ward has an antenatal screening test for sickle haemoglobin. D. D. D. Commonce chemotherapy straight away. Sickle cell trait. and Hb -electrophoresis shows an HbA band only with HbA2 quantitation of 4. C. a bone marrow aspirate confirm that he has acute lymphoblastic leukaemia. C. E. Sickle-B-thalassaemia. B. B-thalassaemia trait. 151 . Skin necrosis. Thrombocytopaenia. B. Discharge him and review in 1 week time as an out patient.8%. 95. Sickle cell diseas(HbSS). E. B. C. This is positive deposite a norm al Hb & MCV. Osteoporosis. The Hb-electrophoresis shows HbA 55% and Hbs 45%.PEDIATRICS EXAMINATION REVIEW 94-A 5 years old presents to casuality with a 2 months history of inguinal lymphadenopathy. Which is the most appropriate initial course of action ? Options: A. Haematologically normal. Peripheral neuropathy. E.Which of the following is not a recognised complication of anticoagulation therapy? Options: A.

Na=137 K=4.4 urea=10.Stomach distension increases colonic motility. C. presence of postural hypotension. on examination he has flacid weakness predominantly in the legs and absent reflexes. which one of the following statement(s) regarding enuresis is not true? Options: A. 152 .55 1. D.1 . Henoch-Schonlein purpura.CH50= 87% (50-110) Urinalysis: blood 3+.PEDIATRICS EXAMINATION REVIEW 97-A 9 years old male has always wet the bed. low grade fever and has some cervical lymphadenopathy. History of diarrhea in the preceding few weeks. Drinking tea causes bed-wetting D. 100-Only one of the following is correct regarding physiology of the colon: Options: A. E. If a child has tried many treatments over a number of years the prognosis is poor. There is a 30% chance that his father was a bed-wetter. D. Wegener’s granulomatosis. E. E. Sever weakness. C. Runs in families. B.creatinin =141 . Mucous. few red cell casts. C. he has no daytime enuresis. B. E. post-streptococcal glomerulonephritis. C. At 15 years age. Na and Cl are actively secreted.C3= 1. Ig A nephropathy. D. Which of the following does not imply a worse prognosis? Options: A. exa mination is otherwise unremarkable.31g/l (0. protein +. It is the site of most vitamin C absorption. K and bicarbonate are absorbed.20)C4= 0. 98-A 18 years old student becomes un well with a sore throat. Interstitial nephrosis. Increased parasympathetic activity leads to decreased colonic motility.50). Markedly reduced FVC. B. there is a 1% chance that he will still bed -wet. CSF examination shows markedly raised protein & a mild lymphocytosis.42g/l (0. investigations reveals: FBC normal . Evidence demyelination on nerve conduction studies. on consulting his GP 3 days after the onset of this illness. 99-An adolescent male is admitted with progressive limb weakness.20-0. What is the most likely diagnosis? Options: A. B.

He 153 .A 14-year-old boy is rescued from a burning building and is brought to the hospital.PEDIATRICS EXAMINATION REVIEW Answer &Teaching Notes for Questions (PART 2) 1.

diarrhoea and abdominal pain. Causes of protein C deficiency are: 1) Congenital * homozygous deficiency . COHb levels of >40% and pregnant women 2-Low protein C levels are not seen with: Options: A. Many people remain asymptomatic. cardiac complications.meningococcal disease and varicella infection * DIC * hepatic disease * drugs . Factor V Leiden refers to a point mutation in the gene for factor V results in resistance of factor V to the actions of activated protein C. A posit ive family history of thrombosis is associated with an increased risk of symptoms .(protein C levels <1% of normal) present with severe neonatal purpura fulminans. * heterozygous deficiency (protein C levels 30 -40% of normal) most frequently presents after puberty with deep venous thrombosis of the lower limb. The indications for hyperbaric oxygen are neurological or psychiatric symptoms. High flow oxygen via a facial mask C. Neonatal purpura fulminans C. It can also present with recurrent superficial thrombophlebitis and DVT. cyclophosphamide. Hyperbaric oxygen B.those with severe deficiency .50% of individuals with heterozygous protein C deficiency and a family history of thrombosis. 5 -fluorouracil). and consequently a thrombophillic state. methotrexate. Meningococcal septicaemia C Teaching Notes for Question 2 Theme: Low protein C levels Protein C is a vitamin K -dependent protein synthesised in the liver. Chicken pox E. Warfarin therapy B. It circulates in an inactive form.PEDIATRICS EXAMINATION REVIEW has nausea. Activated protein C inhibits coagulation by degrading coagulation factors Va and VIIIa and resulting in decreased thrombin formation. He is confused. Which of the following is the best treatment for this patient? Options: A. Intravenous fluid and oxygen via nasal prongs E. chemotherapeutic agents e. Protein C levels are not decreased 154 .+ . will experience a thrombotic event. They can present with massive venous thrombosis as older children. 2) Causes of acquired protein C defic iency: * infections . lasparaginase * malignancy .acute myeloid leukaemia * chronic inflammation . Factor V Leiden D. cerebral thrombosis and DIC. pregnancy and the oral contraceptive. Those with milder defects have protein C le vels of 10 -24 % of normal. CT brain and lumbar puncture D. It requires protein S as a cofactor. which becomes activated by thrombin/thrombomodulin complexes on the surface of endothelial cells. vomiting.inflammatory bowel disease * haemolytic uraemic syndrome * thrombotic thrombocytopaenic purpura Patients with protein C deficiency are at high risk for warfarin -induced skin necrosis during initiation of therapy with warfarin. Urgent senior surgical opinion to exclude bowel obstruction A Teaching Notes for Question 1 Theme: Carbon monoxide poisoning Carboxyhaemoglobin (COHb) levels do not correlate well with the clinical picture. Most thrombotic episodes occur spontaneously but known associated risk factors include surgery with immobilisation. The carboxyhaemoglobin (COHb) level is 25%.warfarin.g.

Anti-smooth muscle antibodies in Primary biliary cirrhosis C. Endothelial cell C.Anti liver/kidney microsomal antibodies (LKM -1) Anti liver cytosol Primary Biliary Cirrhosis . Anti mitochondrial antibodies type II . Anti smooth muscle antibodies.PEDIATRICS EXAMINATION REVIEW 3-Choose the correct matching pair of dis ease and antibody out of the following options: Options: A. Post-streptococcal glomerulonephritis C. IgA nephropathy (Buerger’s disease) E Teaching Notes for Question 5 Theme: Renal disease and complement levels Primary membranous nephropathy and Buerger’s disease can have normal complement levels. Anti liver cytosol antibodies in primary sclerosing cholangitis C Teaching Notes for Question 3 Theme: Antibodies in GI diseases Autoimmune hepatitis type I . 5-Which of the following conditions with renal involvement is the most likely to have normal complement levels: Options: A. Type I pneumocytes. which are as many as type II pneumocytes. Its key function is gaseous exchange. Type I pneumocyte E. 155 . cover 95% of the epithelial surface area. Type II pneumocyte E Teaching Notes for Question 4 Theme: Surfactant production Type II pneumocytes produces surfactant in the lungs. Goblet cell D. Anti-liver/kidney microsomal antibodies (anti LKM-1) in type I autoimmune hepatitis B. Anti-mitochondrial antibodies in type II autoimmune hepatitis E.Anti nuclear antibodies.ANCA positive . The other conditions are all associated with activation of the classical complement pathway and immune complex formation . Anti-nuclear antibodies in Primary biliary cirrhosis D. 4-hich one of the following cells in the lung parenchyma produces surfactant? Options: A. Type II cryoglobulinaemia E. Alveolar macrophage B. Grade IV lupus nephritis B. Subacute bacterial endocarditis D. Anti mitochondrial (M2) antibodies Primary Sclerosing cholangitis .Anti nuclear antibodies.

Visceral leishmaniasis is endemic in parts of Spain . 7-The best treatment option for a patient with Usual Interstitial Pneumonia (UIP or CFA (Cryptogenic fibrosisng alveolitis) is :Options: A. Visceral leishmaniasis is not found in the southern European countries. Yellow fever is endemic in India and South -East Asia. Malaria is only endemic in the tropics. but the latter is also found in South America . Cyclophophamide E. Interferon-1-Beta C. Lung transplantation B. Italy and Greece.PEDIATRICS EXAMINATION REVIEW 6-Which of the following statements about the epidemiology of tropical diseases is correct? Options: A. Azathioprine D. Chaga's disease is confined to South America. E. Arabia. C. High -dose steroids Teaching Notes for Question 7 Theme: Usual Interstitial Pneumonia (UIP or CFA ) At present the treatments for UIP have limited success and most patients will die from their disease. 156 . which lie north of the Tropic of Cancer. Northern India and South East Asia . D. The distribution of infection with Schistosoma mansoni and Schistosoma haemato bium are similar. Chagas' disease is found mainly in South America . Single lung transplantation for UIP results in an actuarial survival of 80% at one year and 5 5% at three years. Teaching Notes for Question 6 Theme: Epidemiology of tropical diseases Yellow fever occurs in Africa and South America only . Schistosoma haematobium and S . B. Malaria is endemic in large a reas of the south shore of the Mediterranean.mansoni are both endemic in Africa .

PEDIATRICS EXAMINATION REVIEW 8-What is the diagnosis that can you infer from the X -ray abdomen? Options: A. This is a cytoskeletal associated protein which is required for B . LBO (Large bowel obstruction) B. Chagas Disease E. platelets. What is the most likely underlying diagnosis :Options: A. at risk of perforation Causes: * IBD * Ischaemia * Infection (clostridium) 9-A 6 month old boy with severe eczema presents with easy bruising and two nose bleeds. Ataxia telangiectasia C. In vitro studies with T-cells. He is below the 2nd centile for weight. He has had 3 previous admissions to hosp ital with pneumonia and has required two courses of antibiotics for otitis media.and T. Wiskott Aldrich syndrome Teaching Notes for Question 9 Theme: Severe eczema and bruising Wiskott Aldrich syndrome is an X-linked combined immunodeficiency caused by a deficiency of WASP (Wiskott Aldrich syndrome associated protein). Toxic Megacolon D. Adenosine deaminase deficiency D. Primary antibody deficiency B. Ankylosing Spondylitis Teaching Notes for Question 8 Theme: Toxic megacolon Large bowel thick walled and di lated. and phagocytes of WAS patients show defects in the 157 . C4 deficiency E.cell interaction and platelet function. SBO (Small bowel obstruction) C.

Characterised histologically by tubule dysplasia and cystic tissue throughout the renal parenchyma C. normal levels of IgG and high levels of IgA and IgE. Initially lymphocyte numbers are normal. autoantibodies bind to acetylcholine receptors at the neuromuscular junction. thrombocyt opaenia and recurrent bacterial infections. Patients present with progressive neurological deterioration.structures which are affected by actin reorganisation. They also have an increased risk of lymphoid malignancy. There is impaired cell mediated immunity (with lymphopen ia) and a characteristic pattern of immunoglobulin levels:. ataxia. Hyperacute rejection is usually due to preformed anti -donor antibody. Patients present with eczema. Myasthenia gravis. Host antibodies (usually IgM) react with foreign antigens on the incompatible transfused blood cells and lead to complement-mediated haemolysis of these cells. Usually fatal at term B. impaired cell mediated immunity and defective antibody production. Later lympho cyte numbers decrease. Antibody . There is also reduced expression of CD43 (sialophorin) on the surface of B and T-lymphocytes. drug-induced thrombocytopenia and hyperacute graft rejection are all examples of type II hypersensitivity reactions.PEDIATRICS EXAMINATION REVIEW formation of microvilli and phagocytic vacuoles . Ataxia telangiectasia is caused by defective DNA repair mechanisms. These children can present with life threatening bleeding. This interferes with normal motor end plate function and also results in a reduction in receptor numbers. Blood transfusion reactions exemplify type II hypersen sitivity. Patients have recurrent respiratory infections and are susceptible to organisms such as Pneumocystis carinii and herpes simplex virus. The Arthus reaction is a type III hypersensitivity reaction mediated by the local dep osition of immune complexes with consequent complement activation and neutrophil infiltration. Hyperacute graft rejection E. Kown to exist in 4 genetic forms 158 . Quinidine-induced thrombocytopenia C Teaching Notes for Question 10 Theme: Type II hypersensitivities. or promoting phagocytosis of antibody-coated cells. Characterised histologically by micro .and macroscopic cysts distributed throughout the renal parenchyma E. They can also bind to cell surface receptors interfering with normal function. Adenosine deaminase defiency causes about 20% of cases of SCID. Haemolysis following transfusion of blood bearing mismatched blood group antigens C.low IgM. Bone marrow transplant is curative. malignant disease. The Arthus reaction D. Production of autoantibodies to acetylcholine receptors in myasthenia gravis B. autoimmune haemolysis. Platelet numbers are decreased and platelets are smaller than usual. In this disease. Type II hypersensitivity reactions involve antibody-mediated destruction of cells. These antibodies are directed against foreign MHC molecules of other alloantigens 11-Polycystic kidney disease is: Options A.usually IgG or IgM can destroy ce lls by activating complement. and the major defect is impaired production of antibody to thymus independent polysaccharide antigen. Always clinically apparent at birth D. An example of antibody interfering with normal receptor function occurs in myasthenia gravis. and immunodeficiency becomes more severe. 10-All of the following are type II hypersensitivities except: Options: A.

E. abscesses) (iv) Tuberose sclerosis (v) Sturge-Weber syndrome (vi) Hypo. The radiologist reports the presence of intracranial calcification. Treatment with pyrimethamine and sulphadiazine is recommended during the first trimester to reduce risk of foetal infection. microcephaly and intracerebral calcification with psychomotor retardation. today in the absence of severe pulmonary hypoplasia affected infants can be expected to survive beyond the neonatal period. CMV. 12-Which of the following statements regarding toxoplasmosis in pregnancy is false ? Options A. Although foetal infection risk is greater during late gestation. * Neither type is always obvious at birth. Documented seroconversion confirms infection. Subdural haematoma B. Ataxia telangiectasia E. B. Infection in the mother is usually asymptomatic. D. astrocytoma) (viii) Vascular lesions Ataxia telangiectasia is not generally associated with intracrania l calcification 159 . Teaching Notes for Question 12 Theme: Toxoplasmosis in pregnancy. Foetal abnormality may occur in cases of maternal infection occurring 6 months pre -conception. Risk of fetal infection is increased with increasing gestational age. the consequences of infection are greater during early pregnancy. but not renal dysplasia. * Histologically it is characterised by the presence of cysts. 13-A 9-year-old has a cranial CT scan. the recessive form not being apparent often until adulthood. Hypoparathyroidism Teaching Notes for Question 13 Theme: Intracranial calcification Causes of intracranial calcification: (i) Choroid plexus calcification (ii) Secondary to intracranial bleed (iii) Infectious causes (toxop lasma. maculopapular rash and hepatomegaly.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 11 Theme: Polycystic kidney disease * This c ondition is generally classified as autosomal dominant or recessive. and nor is either form universally fatal. Toxoplasmosis D. Which of the following diagnoses is least likely to be associated with the radiologist’s report? Options: A. Features of congenital infection include choroidoretinopathy. Pyrimethamine is contraindicated during the first trimester. Sturge-Weber syndrome C. chronically IgM positive with low levels lasting up to 2 years post -infection. The neonate may present acutely with petechiae. C. Infected women may be ‘serofast’.and hyperparathyroidism (vii) Tumours (craniopharyngioma. TB. Maternal toxoplasma-specific IgM may persist for over a year.

15-Regarding Histiocytosis X :Options: A. Noonan's syndrome B.Bochdalek hernia . abdominal organs and in right sided hernias.hiatus hernia. and in left sided hernias. It is a rare cause of pneumothorax Teaching Notes for Question 15 Theme: Histiocytosis X This is a rare disease (prevalence 1/50000) affecting lung. Down's Syndrome D. It is always symptomatic at birth E. there is decreased left sided air entry with hea rt sounds heard on the right.liver into the thoracic cavity.PEDIATRICS EXAMINATION REVIEW 14-Choose the one correct statement regarding a Congenital diaphragmatic hernia: Options A. bone and anterior pituitary B. Malrotation is also seen as part of this condition. The usual presentation is with severe respiratory distress soon after birth. Treatment is with active resuscitation followed by surgery. cardiovascular and CNS anomalies and Trisomy 21. It usually presents with vomiting Teaching Notes for Question 14 Theme: Congenital Diaphragmatic Hernia Congenital diaphragmatic hernia refers to the herniation of abdominal contents into the thoracic cavity. Clinical presentation is varied from unifocal bone lesions to widespread multiple small lung cysts and nodular shadowing. A few infants may have a delayed presentation with vomiting as a re sult of intestinal obstruction or mild respiratory distress. Rapidly worsening progressive disease is treated with etopiside. William's syndrome C. It is a cystic disease of lung. Other associated anomalies include omphalocele. It is most commonly seen on the right side B.Morgagni hernia . It is indeed a ra re cause of pneumothorax. It is associated with pulmonary hypoplasia C. 80% of patien ts are smokers. Edward's syndrome 160 . CXR shows small lungs with prominent markings and honeycombing C. 16-A girl with an unusual facial appearance is found to have coarctation. most are men 20-40 years old D. Turner's syndrome E. Possible diagnoses are: Options: A. Pulmonary hypoplasia is associated and is believed to be a causative factor. It is associated with oligohydramnios D. bone and posterior pituitary function.hernia through the anterior foramen of Morgagni . Most important treatment strategy is steroids E. The abdomen is scaphoid.the most common defect is through the posterolateral foramen of Bochdalek (left sided -90%) . There is herniation of the small and large bowel. Congenital diaphragmatic hernias comprise three types: .

Noonan’s syndrome is associated with pulmonary stenosis and HOCM. a slowing of ageing can be induced by caloric restriction even in mammals and by free radical scavengin g. pa rticularly by reactive oxygen species. William’s syndrome has a ssociated supravalvular aortic stenosis. Noonan’s syndrome has a phenotype similar to Turner’s. The median and interquartile range is a good way of summarising the data Teaching Notes for Question 17 Theme: Skewed distribution This is a negatively skewed distribution which is shown above with tail towards the negative side. Teaching Notes for Question 18 Theme: Mechanisms of ageing Ageing is complex and the mechanism unclear. B. D. C. A Normal distribution is best summarised by quoting the mean (for its middle point) and standard deviation (for its spread). thus meaning equal sex distribution. Ageing can be slowed in animal models by free radical scavengers. Ageing is thought to be due to shortening of the telomeres. Facial features of Turner’s syndrome include: webbed neck. The median is greater than the mode E. In addition. In general. Median is in the middle Best way of summarising skewed data is to give the median and interquartile range. but is AD. E. Studies in invertebrates have identified genes 161 . Which is the best conclusion to draw from the data? Options: A. A skewed distribution is best summarised by quoting the median (for its midd le point) and interquartile range (for its spread). 17-A study measures the heights of 13 -yr-old children. In animal models. Ageing is caused by nitric oxide free radical-induced mitochondrial damage. It is thought to occur due to superoxide induced damage to structures such as mitochondria. This has been disputed by the finding that mitochondria from the elderly brain are functional. high -arched palate and low posterior hairline.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 16 Theme: Unusual facial appearance and aortic coarctation Turner’s syndrome is assoc iated with aortic coarctation. it is always good to remember that: 1. The data are normally distributed C. The data are positively skewed B. not coarctation. Ageing is associated with altered insulin signalling. Ageing can be reduced in mammals by caloric restriction. Mode is towards the hump 2. 18-Which of the following hypotheses on the mechanisms of ageing is least accurate? Options: A. The mean is the best measure of central tendency D. Mean is towards the tail 3.

A recent study using knockout mice implicates the mammalian insulin-like growth factor receptor as an important regulator of lifespan. Left lower lobe consolidation B. it is consolidation. 162 . Examples include DAF-2 (caenorhabditis elegans) and InR (Drosophila). left lower lobe. therefore. Left lingular collapse E. * Left paraspinal line (and left hemi -diaphragm) obscured. Left lower lobe collapse C. 19-What is the diagnosis? Options: A.PEDIATRICS EXAMINATION REVIEW that alter lifespan and which also encode components of insulin signalling pathways. Another prominent hypothesis for ageing is that associated with telomere shortening. therefore. Left lingular consolidation D. Right lower lobe consolidation Teaching Notes for Question 19 Theme: Lower lobe consolidation * No loss of volume and there is airspace shadowing.

MPGN 4. A gene whose product is involved either in transforming cells in culture or in inducing can cer in animals is called a proto-oncogene. Membranoproliferative glomerulonephritis B. 21-Which of the following renal disorders is associated with normal serum complement levels? Options: A. Shunt nephritis 5. Primary hyperaldosteronism C. Ehlers Danlos syndrome 163 . Anorexia nervosa E. Teaching Notes for Question 20 Theme: Cancer genetics No teaching note available.2 mmol/l HCO3 36 mmol/l Ca 2. All forms of lupus nephritis C. A proto -oncogene is an abnormal gene that encodes a protein involved in regulation of cell growth or proliferation and that can be mutated into a cancer -promoting gene. and BRCA1 are oncogenes C. Type II cryoglobulinemia Both infective endocarditis and shunt nephritis are immune complex mediated disorders with activation of the complement pathway and resul ting hypocomplementemia. Lupus nephritis 3. E. rapidly progressive GN is a clinical diagnosis and is not necessarily hypocomplementemic. Shunt nephritis D.PEDIATRICS EXAMINATION REVIEW 20-Which one of the following is true regarding cancer genetics?: Options: A. Causes of low C3 levels include: 1. Any gene whose encoded protein directly or ind irectly inhibits progression through the cell cycle and in which a loss-of-function mutation is oncogenic is an oncogene.4 mmol/l P04 1.0 mmol/l Urine chloride 10 mmol/l Options: A. Primary hyperparathyroidism B. Persistent vomiting D. Goodpasture’s disease Teaching Notes for Question 21 Theme: Complement levels and glomerular disease. RB. APC. Infective endocarditis with renal involvement 6. D. B. Post-infectious glomerulonephritis 2. A proto-oncogene is transformed on to an oncogene by changing the protein -coding segment or by altering its expression. Post-infectious glomerulonephritis E. Similarly. Note that Focal segmental glomerulosclerosis is not associated with low C3 levels. 22-What is the most likely explanation for th ese biochemical changes in a 15 -year-old girl with erosive changes in her mouth? Na 138 mmol/l K 3.

It acts as an opsonin enhancing phagocytosis. C5a is a compl ement intermediate which acts as a potent chemokine. May be defined as increased vascular permeability accompanied by an infiltration of inflammatory cells D. AV reentrant tachycardia due to accessory pathway E. The resulting inflammation: Options: A. The resolution of inflammation is tightly regulated. and also activates the classical complement pathway. The combination of increased vascular permeability and cellular influx gives rise to the calor. CRP has a half -life of about 12 hours. CRP is an acute phase protein which is synthesised by hepatocytes as a result of the acute inflammatory process. rubor and dolor of the inflammatory process. Sinus tachycardia D. C-reactive protein binds to phosphocholine moieties found on microbial polysaccharides. a potent chemokine B. Ectopic atrial tachycardia 164 . it follows a stereotypical cascade. Causes the release of CRP. Causes the release of C8a. Acute infection results in activation of complement. indicating volume depletion.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 95 Theme: Acid-base disorders She has a metabolic alkalo sis with hypokalaemia and a low urine chloride. Atrial flutter B. It is secreted in increased amounts within 6 hours of an acute inflammatory stimulus. Supraventricular tachycardia C. Is always harmful E. 23-A patient presents with acute infective arthritis. which is not regulated Teaching Notes for Question 23 Theme: Acute inflammation. th e half-life of which is several days C. 24-What does this ECG of an 8-year-old boy with Ebstein’s anomaly show? Options: A. Once initiated.

In addition to arthropathy and obstructive nephropathy. blast crisis of leukaemias. severe psoriasis. Endogenous uric acid comprises roughly two thirds of total body urate. Causes are: -Renal insufficiency -Acidosis .Lesch-Nyhan syndrome . Overproduction of uric acid: This accounts for only a minority of patients presenting with hyperuricaemia.diabetic ketoacidosis. decreased tubular secretion. these patients develop a neurological disorder that is characterised by choreoathetosis. -Diuretic therapy . -Increased purine nucleotide breakdown .results in accelerated hepatic breakdown of ATP and generation of organic acids that compete with urate for tubular secretion. Lesch-Nyhan syndrome B.inhibition of uric acid secretion. dissociated atrial and ventricular activity is never seen as both atria and ventricles are part of the same circuit. However the typical P wave morphology and rate is pathognomonic of atrial flutter. or increased renal tubular reabsorption. cytotoxic therapy -Enzymatic defects . increased cell death rhabdomyolysis. Combined mechanisms: -Alcohol consumption . -Low-dose aspirin . and VII can result in hyperuricaemia due to excessive degradation of skeletal muscle ATP. Accumulation of organic acids competes with uric acid for renal tubula r secretion. while the res t is eliminated by the intestines.g.an enzyme that catalyses the conversion of hypoxanthine to inosinic acid. Low-dose aspirin is associated with hyperuricaemia. Approximately 70% of the urate produced daily is excreted by the kidney. Thiazide diuretic therapy C. Causes are: -Increased intake in the diet. * The next most likely diagnosis with this ECG is ectopic atrial tachycardia.due to rapid cell proliferation e. Hyperuricaemia may be due to: -Decreased excretion (under-excretors) -Increased production (over-producers) -A combination of these 2 mechanisms Decreased uric acid excretion: This can result from a decreased glomerular filtration. Polycythaemia rubra vera D. High-dose aspirin therapy Teaching Notes for Question 25 Theme: Hyperuricaemia Uric acid is the final product of purine metabolism. The remaining one third is derived from the diet. 165 . 25-Which of the following conditions is not associated with hyperuricaemia : Options: A. ethanol. starvation ketosis. * In other SVTs (including those utilising accessor y pathways). -Diabetes insipidus .PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 24 Theme: Atrial flutter * This ECG shows classical saw -toothed atrial waves typical of atrial flutter. The deficiency of HGPRT accelerates purine biosynthesis with a resultant increase in uric acid production.an inherited X-linked disorder due to a deficiency of HGPRT . -Glycogenoses types III. The ventricular rate is typica lly 150/min because of 2:1 AV conduction. whereas high-dose aspirin can have a therapeutic effect in gout. Severe exfoliative psoriasis E.enhanced renal tubular reabsorption of uric acid.enhanced renal tubular reabsorption of uric acid. IV. These are negative in the inferior leads and have a rate of 300/min. spasticity and occasionally self-mutilation.

Hungry bone syndrome is associated with reduced serum magnesium Teaching Notes for Question 26 Theme: Hypomagnesaemia.PEDIATRICS EXAMINATION REVIEW 26-In a patient with hypomagnesaemia.a family history of a first-degree relative diagnosed with NF -1. Prolonged vomiting is an indication for high -dose oral magnesium supplementation E.skeletal manifestations and . the following statement is not true: Options: A. It can be prevented by the use of oral antibiotics Teaching Notes for Question 28 Theme: Necrotising enterocolitis Necrotising enterocolitis is the most common serious surgical condition affecting neonates. Recent cisplatin treatment could have been the cause B. Café au lait patches Teaching Notes for Question 27 Theme: Neurofibromatosis -1 * NF-1 is an autosomal dominant neurocutaneous disorder affecting approximately 1 in 5. A number of factors have been implicated in the aetiology of NEC. Hypomagnesaemia results in defective PTH secretion and secondary hypercal caemia.café au lait patches. 166 . It usually involves the lower colon B.Lisch nodules in the eye. Hypocalcaemia may occur C. Gas in the biliary tree although a pathognomonic sign of NEC. bacterial overgrowth. It may lead to intestinal obstruction a few weeks later E. Neuromuscular symptoms are similar to those in hypocalcaemia D. is not frequently seen. It usually involves the terminal ileum and ascending colon. Lisch nodules E. It is not seen in babies who haven't been started on feeds yet D. The main features of this condition include: . . It is predominantly seen in preterms. rapid introduction of feeds and the use of formula feeds. . Axillary freckling B. Diagnosis of this condition is mainly done on a cl inical basis with gene testing offered to a small minority. Cortical tubers D. includi ng luminal substrate. .axillary freckling. .000 individuals. It is commonly associated with gas in the biliary tree C. Tibial pseudoarthrosis C. 27-All these are major clinical features of neurofibromatosis -1 (NF-1) except: Options A. * Cortical tubers are seen on neuroimaging in another AD neurocutaneous condition called tuberous sclerosis 28-Choose the one correct statement regarding necrotising enterocolitis: Options: A.neurofibromas.

and the protease alleles are co dominant. Hepatic pathology is also associated with tissue damage secondary to the accumulation of polymerised AAT. can be explained by the fact that the liver cells in the infants are less capable of degrading the polymerised protein. 167 . Alpha 1-antitrypsin (AAT) is a serine protease inhibitor (serpin) that protects the connective tissue of the lungs from elastase . PiSZ) have a less severe phenotype. this is not due to a lack of synthesis – rather a blockage of its processing and secretion from the liver. Other members of the serpin family include C1 -esterase inhibitor. this may never develop or occur later in life. Approximately 5% of patients die of their liver disease. and patients with the ZZ phenotype have levels of AAT less than 20% of normal and develop severe disease. Some neonates with the PiZZ genotype may develop rapidly progressive liver damage . and approximately 10–15% of adult patients will develop cirrhosis usually over the age of 50 years. Over 100 different variants of alpha 1-antitrypsin have been identified. The low levels of the enzyme in deficiency syndromes are due to lack of synthesis of the variant enzyme E. The consistent occurrence of overt liver disease in newborn infants. It is a membrane bound tyrosine kinase B. Emphysema in deficiency syndromes is characterised histologically by inclusion bodies Teaching Notes for Question 29 Theme: Alpha1-antitrypsin (AAT) Alpha 1-antitrypsin deficiency is an inherited condition caused by a defective gene on chromosome 14. Heterozygous states (PiMZ. and brain-specific neuroserpin. PiMS. The majority of patients with clinical liver disease are homozygotes with a PiZZ phenotype.Choose the correct statement regarding alpha 1 -antitrypsin (AAT): Options: A. hepatic pathology is often limited to minor portal fibrosis but frequently progresses to overt liver damage in late adulthood. Initially.PEDIATRICS EXAMINATION REVIEW However. In alpha 1-antitrypsin deficiency. Although plasma levels of the enzyme are low. The variability in the severity and age of onset of liver disease among patients with AAT deficiency can be explained in part by individual variations in episodes of inflammation and hence to increased synthesis of AAT. Another allele (Z) results in an increased risk of emphysema. People with both PiS genes (PiSS) are less severely affected. Over 75% of cases of emphysema in the UK are associated with the deficiency of this enzyme C. Deficiency presents as cirrhosis in children D. In non -smokers. The normal allele (M) is seen in over 95% of Europeans. Alpha 1-antitrypsin deficiency can produce jaundice and liver problems in babies within days of their birth. Alpha 1-antitrypsin is encoded by the protease inh ibitor (Pi) locus. The use of oral antibiotics does not reduce the risk of developing NEC. 29.a clinical indication for liver transplantation. AAT deficiency accounts for less than 1% of cases of COPD in the UK. Inclusion bodies in hepatocytes are a feature of AAT deficiency. in contrast to its only occasional occurrence in young adults. with alpha 1 -antitrypsin levels 60–70 per cent of normal. alpha 2 -antiplasmin.an enzyme released by leucocytes during phagocytosis. it may also occur in babies who have never been fed. A few children with alpha 1 -antitrypsin deficiency develop significant liver disease before the age of 20. emphysema results from the uncontrolled action of proteases on pulmonary tissue. Cigarette smoking increases the number of neutrophils in the alveolus and ina ctivates the remaining small amounts of antiprotease. Smoking is the greatest risk factor for the development of emphysema in adults with alpha 1-antitrypsin deficiency.

Crohn’s disease B. It is caused by idiopathic degeneration of parasympathetic neurones in the ciliary ganglion and therefore exhibits denervation supersensit ivity: the affected pupil will constrict on exposure to low concentration of pilocarpine (0. ALP 320 MCV 108 Bili 12. It exhibits X-linked inheritance pattern C. WCC 7. TFTs normal What is the underlying diagnosis ? Options: A. Both her sisters (age 14 and 19) are greater then 170cm tall and are well. PO4 0. With time the affected pupil may become small. she also has macrocytic anaemia. It is associated with early -onset REM sleep E. She is 145 cm tall and weighs 48 kg. Ulcerative colitis Teaching Notes for Question 31 Theme: Primary amenorrhoea This young woman has a classical complication of celiac: Vitamin D deficiency resulting in hypocalcaemia. Night time insomnia can be a feature D.7 . Cystic fibrosis D.PEDIATRICS EXAMINATION REVIEW 30-Which of the following is not a feature of the Holmes –Adie syndrome? Options A. The affected pupil is always larger than the unaffected B. There will be a compensatory rise in her parathyroid hormone levels and there is a risk of secondary hyperparathyroidism. AST 34 Ca2+ 1. Global areflexia is a recognised association E. Both pupils may be affected C. It is associated with cataplexy B. Coeliac disease C.5 . Hb 10. again suggestive of the diagnosis.9 U&E normal.125%) whereas the unaffected will not. Addison’s disease E. There is d enervation supersensitivity of the pupil D. the other pupil may also become affected 31-A 17 year old girl presents with primary amenorrhoea.6 . It may be associated with hypnopompic hallucinations 168 . She has recently started developing breasts. Ptosis is rare Teaching Notes for Question 30 Theme: Holmes -Adie syndrome Holmes-Adie syndrome consists of impaired pupillary constriction to light associated with loss of deep tendon reflexes. 32-Which of the following is not true of narcolepsy? Options: A.

blurred vision and even pain in the throat are warning signs. which occurs once per week or per month and is usually associated with a strong family history. vivid hallucinations on falling asleep (hypnogogic) or waking (hypnopompic). In many cases headache is associated with photophobia. Migraine is an acute headache. FISH analysis is used as a second line of investigation after conventional light microscopy. More than 50% of headaches are unilateral B. and sleep paralysis. Chronic. EEG will help in the diagnosis C. The headache may last from one hour to 2 –3 days. Sumatriptan is not licensed for use in children Teaching Notes for Question 33 Theme: Migraine Headache is one of the commonest reasons for referral to the neurology clinic. Sleep studies show abnormal periods of early -onset REM sleep. There is a breed of Doberman pinschers in whom the condition is dominantly inherited (AD). 169 . More than 50% of cases are not unilateral. Triptans are not licensed in children but various studies have proven their efficacy in adults. Very small rearrangements can be identified by this technique. the affected gene encodes a protein produced in the lateral hypothalamus called hypocretin. blurred vision and vomiting.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 32 Theme: Narcolepsy This idiopathic condition is characterised by excessive daytime sleepiness associated with cataplexy (loss of muscular tone triggered by emotional events). The acute headache. Sleep in a dark quiet room with analgesia is often useful. non-progressive headache needs non -urgent investigation and initial treatment with simple analgesia can be commenced. An aura and postdrome stage is present as in adult patients E. A history of stomach pain. 34-The following statements about techniques using FISH (fluorescent in situ hybridisation) are true except: Options: A. The technique can be performed on uncultured cells B. which presents over a short period of time with neurological deficit. Special microscopes using UV filters are used to detect the fluorescent signals. The technique requires specialised microscopes C. The technique can detect small deletions in DNA D. Interphase FISH on uncultured cells is currently used in rapid prenatal diag nosis. The technique can be used to map the position of genes on a chromosome Teaching Notes for Question 34 Theme: Diagnostic techniques Artificially generated DNA probes carrying a fluorescent label hybridise (attach) to known specific chromosomal regions. and usually the classical picture of migraine attack does not accompany the attack. Sometimes a multi -disciplinary team approach is required. requires urgent investigation. Sleepiness responds to stimulants and cataplexy to clomipramine. 33-The following are all true about migraine in children except: Options: A. One of the main research uses of FISH is to localise genes on chromosomes. The technique is used instead of light microscopy for routine chromosome analysis E. Other members of the family may be affected D.

(NB. Pneumococcal pneumonia D. but are short lived cells in order to limit antibody responses. Production of immunoglobulin with higher affin ity to the antigen C. Isotype switching to IgG B. Memory B -cells differ qualitatively as well as quantitatively from naïve B cells. Tuberculosis B. Rheumatoid arthritis E. but rheumatic fever does not lead to tamponade that needs decompression. Uraemia Teaching Notes for Question 35 Theme: Cardiac tamponade All the above cause pericarditis. A quantitatively increased antibody production as compared to the primary immune response E. Certain bacterial antigens can activate B cells directly. They secrete large amounts of anti body. the frequency of antigen -specific B-cells may increase 10-100 fold. Memory B -lymphocytes have undergone class switch and produce mainly IgG but also other immunoglobulin isotypes. After priming during t he primary immune response. and larger amounts of antibody are produced. Requirement of help from T-cells Teaching Notes for Question 36 Theme: Secondary immunoglobulin response Memory B-cells are responsible for the secondary Ig response. Plasma cells are the effector cells of both primary and secondary immune responses. Therefore the secondary immune response to a specific antigen occurs more rapidly than the primary response. 36-The secondary immunoglobulin response is not characterized by: Options: A.g. Both prim ary and secondary immunoglobulin responses require T-cell help. 170 .PEDIATRICS EXAMINATION REVIEW 35-Cardiac tamponade may occur with all of the following except: Options: A. e. Memor y B-cells also have higher affinity for antigen as they have undergone affinity maturation in the germinal centres of lymphoid tissue. LPS). Production of memory plasma cells D. Rheumatic fever C.

Myelomatous deposit E. bronchiectasis. In multiple endocrine neoplasia type 1. Serum C -peptide will not be raised in exogenous insulin use E. 171 . Osteopetrosis Teaching Notes for Question 37 Theme: Hypertrophic Osteoarthropathy HPOA . levels of glucose. AVN (Avascular necrosis) talus D. pituitary. pancreas. and parathyroid tumours occur. glucose and C-peptide should be taken every morning for 7 days D. dysentery. coeliac. ideally. Causes of hypoglycaemia include insulinoma. Serum insulin and urine sulphonylureas levels help check for use of these drugs. lymphoma.PEDIATRICS EXAMINATION REVIEW 37-What is the abnormality seen in this girl with bone pain? Options: A. Whipples.periosteal reaction along distal long bones. abscess. A 72hr fast should be organised. lymphoma. Serum insulin. HPOA (Hypertrophic Osteoarthropathy) C. Subperiosteal resorption of HPTH (Hyperparathyroidism) B. with the doctor called when the blood sugar reaches 4mmol/l. A family history of scars in the neck is irrelevant Teaching Notes for Question 38 Theme: Hypoglycaemia Investigation of hypoglycaemia requires a 72hr fast. metastasis * Pleural: fibroma or mesothelioma * CVS: Cyanotic congen heart disease * GIT: IBD. cirrhosis esp PBC Subperiosteal resorption – HPTH. Urine sulphonylurea levels are unhelpful B. C-peptide and insulin are taken when BM is LOW <4. 38-In the investigation of a patient who is admitted following 3 dizzy spells each markedly improved by eating:Options: A. Causes: * Chest: CA bronchus. C.

In cases of exogenous insulin administration. Also check ESR and complement levels. 172 . neck and hands. plasma insulin and C -peptide are high. In insulinoma. Elevated levels produce gynaecomastia without galactorrhoea B. injury to the stalk. What are the most appropriate tests to request? Options: A. Levels are increased by cimetidine Teaching Notes for Question 40 Theme: Prolactin Elevated prolactin levels produce gynaecomastia with galactorrhoea. The cutaneous lesions of SLE can be sub -divided: i) Mauve erythema over cheeks and nose: Butterfly rash ii) Maculopapular erythematous rash on sun -exposed skin iii) Chilblain lupus iv) Vasculitis v) Diffuse alopecia vi) Raynaud’s phenomenon vii) Livedo Reticularis (light purple net -like rash on limbs) viii) Urticarial eruptions ix) Bullous lesions from photosensitivity x) Discoid Lupus (Chronic Cutaneous Lupus) xi) Annular erythema in photosensitive distribution (Sub -acute Lupus Erythematosus) xii) Generally. Metoclopramide is a dopamine antagonist. increased TRH (as TSH decreased) and co -secretion of prolactin mean that 30% have prolactin elevation. and urine sulphonylurea levels are undetectable.PEDIATRICS EXAMINATION REVIEW exogenous insulin administration. which increases pro lactin levels. Levels are elevated in >90% of cases of acromegaly E. Cimetidine has a weak anti-androgen effect. and urine sulphonylurea levels are undetectable. C-peptide is not elevated. Full blood count and chemistry D. c-reactive protein and erythrocyte sedimentation rate C. 40-Which of the following is true about prolactin? Options A. antiDNA antibodies. In cases of sulphonylurea -induced hypoglycaemia. Damage to the pituitary stalk reduces prolactin levels C. Metoclopramide increases prolactin production D. Prolactin secretion is inhibited by dopaminergic stimulation carried in the pituitary stalk. and sulphonylurea intake. Anti nuclear antibody and c-reactive protein B. which produces gynaecomastia. but not galactorrhoea or e levated prolactin. Check ANA. any rash in a photosensitive distribution should have SLE excluded. and sulphonylurea can be detected in urine. She attributed the rash to severe sunburn and says that she has sensitive skin. joint aches and a red rash on her face. Anti-nuclear antibody and erythrocyte sedimentation rate E. 39-A 16-year-old girl presents with fever. plasma insulin is very high. In acromegaly. Complement levels Teaching Notes for Question 39 Theme: Systemic lupus erythematosus This patient has Systemic Lupus Erythematosus. plasma insulin and C-peptide are high. Damage to this increases prolactin levels.

173 . What is the most useful investigation? Options: A. 42-An 18-year-old man is referred to the outpatient clinic as his general practitioner has found him to have microscopic haematuria. Steroids are the agents of first choice. By the age of 9 months a child: Options A. Can stand alone D. 43-The following statement is false regarding treatment of Juvenile Idiopathic Arthritis (JIA):Options A. deafness. He also has bilateral sensorineural deafness with a sister and younger brother who are similarly afflicted. Most children begin to stand alone at 12 months. Can crawl C. Diagnosis is by renal biopsy or occasionally skin biopsy. Anti-GBM antibodies D. Audiometry E. Serum creatinine C. Important for discussion about inheritance.PEDIATRICS EXAMINATION REVIEW 41-Choose the incorrect statement. anterior lenticonus. ear and eye leading to clinical disease of haematuria. Urine microscopy B. Can sit with support B. which is present in the glomerular basement membrane. Has lost the Moro reflex Teaching Notes for Question 41 Theme: Developmental milestones Developmental milestones reached by 9 months: Gross motor development: * Can sit unsupported for 10 -15 minutes * Can reach out for objects and still maintain balance * Crawling * Pulls to stand Fine motor development: * Starts to grasp object between thumb and forefinger * Bangs two bricks together * No hand preference Social development: * Feed self with biscuit (5 to 8 months) * Responds to name Speech and language: * Babbles with syllables Moro reflex appears at birth and disappears at 4 -5/12 of age. corneal lesions and rarely leiomyomata. Can play peek -a-boo E. Renal biopsy Teaching Notes for Question 42 Theme: Alport's syndrome Only renal biopsy can confirm a diagnosis of Alport's. then proteinuria and progressive renal impairment. Alports is usually (approx 80%) inherited in an X -linked fashion with a defect in the COL4A5 gene (alpha 5 chain of type IV collagen).

pulmonary fibrosis and GI symptoms especially nausea and vomiting. outlines liver due to perforation of an intraperitoneal viscus (not a retroperitoneal viscus). Gold salts may lead to nephrotic syndrome. 174 . 44-What is the abnormality shown on Chest X -ray? Options: A. Perforation E. Low dose methotrexate may be beneficial Teaching Notes for Question 43 Theme: treatment of Juvenile Idiopathic Arthritis Methotrexate is the treatment of cho ice in polyarticular and extended oligoarticular diseases. subcutaneous or intramuscular route. Hydroxychloroquine is associated with ocular toxicity. Consolidation right lower lobe B. Collapse right lower lobe C.Perforation Perforation * Free air under the hemi-diaphragm. Collapse lingular lobe D. E. and can be administered via the ora l. Popular viva question in Part2: MTX is given weekly. C. Iridocyclitis usually responds to topical steroids. D. Bilateral pneumothoraces Teaching Notes for Question 44 Theme: Chest X-ray . intra -articular steroids are usually tried first before systemic treatments. In cases of oligoarticular disease. Side Effects include marrow suppression.PEDIATRICS EXAMINATION REVIEW B.

Often leads to chronic haemolysis. CMV retinitis E. 175 .PEDIATRICS EXAMINATION REVIEW 45-What is the diagnosis? Options: A. The haemorrhages and exudates look too well defined for this to be CMV retinitis. The absence of new blood vessels is against a proliferative retinopathy. C. Endocarditis would tend to produce more discreet white balls on fundoscopy. Teaching Notes for Question 46 Theme: Glucose-6-phosphate dehydrogenase deficiency G6PDH catalyses the synthesis of NADPH from the hexose monophosphate pathway. E. This slide demonstrates retinal blot haemorrhages and exudates. Requires treatment with desferrioxamine to prevent iron overload. Grade 3 hypertensive retinopathy C. Is caused by mutations of the G6PD gene on chromosome 4. Is more common in women than in men. Endocarditis D. Is associated with haemolytic crises induced by sulfonamides. Background retinopathy Teaching Notes for Question 45 Theme: Retinopathy. 46-All of the following statements about Glucose -6-phosphate dehydrogenase deficiency are false except: Options: A. D. Proliferative DM retinopathy B. The absence of microaneurysms is against a diagnosis of background retinopathy. This appearance would do for hypertensive or diabetic retinopathy. The NADPH is then used to reduce glutathione and hence in c onjunction with glutathione reductase reduces cellular oxidative damage. B.

The various mutations lead to altered expression of G6PD or an altered half-life of the enzyme. vitamin K and nalidixic acid. There are a number of disease manifestations: . CD59 or DAF deficiencies result in paroxysmal nocturnal haemoglobinuria. 48-The following autoantibodies are commonly found in the named disease: Options A. chloroquine. Anti-liver cytosol antibodies in primary sclerosing cholangitis Teaching Notes for Question 4 8 Theme: Autoimmune hepatitis Type I autoimmune hepatitis is characterised by the presence of anti -nuclear antibodies. aspirin (high doses). primaquine. Haemolytic crises vary in severi ty. anti-liver 176 . Reduced levels of C3 can also be seen in cholesterol emboli.fava bean. Anti-mitochondrial antibod ies in Primary biliary cirrhosis D. CGD). At least 400 G6PD variants have been identified.naphthalene. It is a balanced polymorphism associated with resistance to falciparum malaria in heterozygous females. anti-smooth muscle antibodies and anti-mitochondrial antibodies. C4) leads to bacterial infections. C2. Hereditary angioneurotic oedema is due to C1 inhibitor deficiency. caused by mutations in the X -linked (Xq28) G6PD gene. post streptococcal glomerulonephritis and mesangiocapillary glomerulonephritis type II. C1q deficiency and angio -oedema C. rarely chronic haemolysis may occur. ANCA in type II autoimmune hepatitis E. G6PD deficiency can cause immunodeficiency by reducing the NADPH required for activation of the NADPH oxidase enzyme (cf. Deficiencies in the components of the membrane attack complex (C5 -C9) result in susceptibility to Neisseria infection only (estimated 10. P) leads to infection with pyogenic bacteria and Neisseria but no immune complex disease.patients may present with neonatal jaundice .C2. C3 deficiency is associated with a wide range of pyogenic infections and Neisseria infections and sometimes with immune complex disease.PEDIATRICS EXAMINATION REVIEW G6PDH enzyme deficiency. Infections can also provoke haemolysis.usually the deficiency manifests as acute haemolytic crises provoked by oxidising stress e. Deficiencies of MBL (MBL.patients may be asymptomatic . affects over half a billion people worldwide. Anti-liver/kidney microsomal antibodies (anti LKM-1) in type I autoimmune hepatitis B. both C3 and C4 are reduced. Anti-phospholipid antibodies in Primary biliary cirrhosis C. C3 def is associated with par tial lipodystrophy E. C2 deficiency and rheumatoid arthritis D. MASP1. certain foods . sulphonamides.g. C4) predispose to immune-complex disease and systemic lupus erythematosis (due to inefficient removal of apoptotic cells).dapsone. MASP2. C5-C9 deficiency and mycoplasma infection B. 47-Which of the following statements correctly describes the clinical pheno types which are associated with a deficiency of the various complement components? Options: A. mainly in childhood. nitrofurantoin. This evolutionary advantage outweighs the small negative effect of affected hemizygous males. A raised C4 and low C3 in SLE is associated with active disease Teaching Notes for Question 47 Theme: Complement Deficiencies in the components of th e classical pathway (C1. Deficiencies in the alternative pathway (Factor D. chemicals . Type 2 is characterised by anti -liver/kidney microsomal antibodies (LKM-1).000 fold!). If severe. In active SLE. certain drugs .

Ceftriaxone can be given through the IO needle.g. severe MR/AR. There is also a ptosis present in this eye. He is not responsive to pain. Give immediate IM ceftriaxone before securing IV access E. his airway is patent and he is breathing oxygen via a mask with non -rebreathing bag with oxygen saturations of 100%. 51-A patient is noted to have a small left pupil. He has been unwell f or 12 hours with fever and has vomited once. The current priority therefore is treatment of his shock (tachycardia and prolonged CRT). Where is the lesion likely to be? Options A. posture. intraosseus access should be secured and fluid resuscitation with colloid administered. Soft aortic component of second heart sound D. Continue to try for IV access and then give 20mL/kg fluid if suc cessful B. It is very difficult to secure IV access when a child is so peripherally shut down. The next step should be: Options: A. and blood sugar). Third heart sound C. Check blood sugar C. Oculomotor nerve B. His heart rate is 160/min. are ABC. A colleague has been trying for 5 minutes to secure intravenous access when you arrive. Secure intraosseus access and give 20mL/kg fluid D. pupils. as always. Primary biliary cirrhosis is characterised by anti -nuclear antibodies and anti-mitochondrial (M2) antibodies. His mother has noted that the left si de of his face is dryer than normal. 49-All of the following are clinical fe atures of severe aortic stenosis except: Options: A. 'disability' can be fully assessed (coma score. Systolic thrill E. Trigeminal nerve 177 . and in a child like this who is unresponsive to pain. On examination. After fluid resuscitation.PEDIATRICS EXAMINATION REVIEW cytosol antibodies. Primary sclerosing cholangitis is characterised by ANCA positivity. Length of murmur in systole Teaching Notes for Question 49 Theme: Features of aortic stenosis 5 clinical signs of severe AS are: * Slow rising pulse character/Narrow pulse pressure * 4th HS * Soft aortic 2nd HS * Systolic thrill * Length of murmur in systole 3rd HS is a feature of the volume -loaded LV e. Loudness of a murmur is usually NOT a clinical feature of its severity. 50-A two-year-old boy is brought to the resuscitation room. Check a capillary blood gas Teaching Notes for Question 50 Theme: Resuscitation in sepsis The priorities. Fourth heart sound B. This child has a patent airway and adequate oxygenation. decompensated heart failure. capillary refill time is 5 seconds and he has a pur puric rash over his lower limbs.

ptosis. Sinking in of one eye D. Vagus nerve E. Immunosupressed people due to disease or treatment B. Cervical sympathetic trunk Teaching Notes for Question 51 Theme: Horner's syndrome Interruption of the cervical sympathetic trunk results in Horner’s syndrome. Those with coeliac disease Teaching Notes for Question 53 Theme: Influenza vaccine Influenza viruses constantly alter there H (haemagglutinin) and N(neuraminidases)antigens present on the surface. a neurysm and dissection). and for people who can spread influenza to those at high ris k (including all household members): People at high risk for complications from influenza: • All children 6-23 months of age. A Horner's consists of ptosis. Possible causes include vascular lesions (carotid pathology). Since these vaccines will not control an epidemic they are only for those people at high risk from infection. Facial nerve D. Especially apical squamous cell carcinoma. trauma and syringomyelia.heart disease . • People 65 years of age and older. Absence of sweating on face and neck E. neck injuries. a syrinx and lung lesions. • Residents of long-term care facilities housing persons with chronic medical conditions . 52-A patient is diagnosed as having a Horner's syndrome following carotid surgery. • People who have long-term health problems with: . Ptosis C. Causes include carotid pathology (tumours. Lack of lacrimation Teaching Notes for Question 52 Theme: Horner's Symptoms of Horner’s syndrome include miosis.PEDIATRICS EXAMINATION REVIEW C. Those with diabetes mellitus E. Every year the World Health Organisation (WHO) recommends which strains should be included. Which of the following is NOT a symptom of Horner’s syndrome? Options: A. 53-Which of the following sub-group of populations is the influenza vaccine not recommended for?:Options A. Those with chronic heart disease C. It is due to an interruption in the symapthetic nerve supply to the eye. Those with chronic renal failure D. pupil constriction and absent sweating over the affected side of the face .It is recommended for people who are at risk of serious influenza or its complications. anhydrosis and enophthalmos. Pupillary constriction B. lung lesions (especially apical squamous cell tumours).kidney disease 178 .

but does increase the degree of AV block – this slows the ventricular rate transiently and may reveal flutter waves on ECG. Other medical conditions. Is usually left atrial in origin B. The alternative hypothesis should be accepted C. and other blood disorders • People with certain conditions (such as neuromuscular disorders) that can cause breathing problems. which can be diagnostic.asthma . The probability of a type I error occuring is 1% D. which can be interrupted by radiofrequency ablation.lung disease . Is often associated with an irregular pulse D. which usually cycles at a rate of 300 bpm (280–320). 54-The lipid-lowering effect of a new statin is compared to atorvastatin. giving an irregular heart rate. The trial would have been better run against placebo Teaching Notes for Question 54 Theme: Data analysis Where the p-value is less than the critical value.metabolic disease. Implies the presence an accessory pathway Teaching Notes for Question 55 Theme: Atrial flutter * Atrial flutter occurs because of an abnormal circuit of current in the atria. The vaccine is an inactivated virus. * Most atrial flutter originates in the RA. such as diabetes . The ventricular rate is determined by the degree of AV nodal block. there is good evidence to accept the alternative hypothesis. 179 . with a counterclockwise circuit around the tricuspid valve annulus. Commonly. giving a ventricular rate of 150 bpm.cancer treatment with x-rays or drugs • People 6 months to 18 years of age on long -term aspirin treatment (these people could develop Reye Syndrome if they got influenza). 55-Which of the following is correct regarding atrial flutter? Options: A. What is the best conclusion to draw? Options A. * Adenosine does not terminate atrial flutter. Although usually regular. Can be terminated with intravenous adenosine E. the degree of AV block sometimes varies from beat to beat. The p-value associated with this statin means that it is a clinically significant new drug E.PEDIATRICS EXAMINATION REVIEW .long-term treatment with drugs such as steroids . Usually causes a narrow complex tachycardia at 150 bpm C. The p -value is the probability of committing a Type I error.HIV/AIDS or other diseases affecting the immune system .001). * The circuit of current usually occurs via an anatomically distinct pathway. including coeliac disea se are not high risk for developing influenza. Critical level is set at 0.01 and difference is found (p = 0. C would be correct if it said 0.1%. • People with a weakened immune system due to: . • Women who will be pregnant during influenza season. every second flutter wave is conducted (2:1 AV block). A larger sample should have been used B.anemia.

PEDIATRICS EXAMINATION REVIEW

56-The following statements are true about Tetralogy of Fallot (TOF) except: Options: A. Children with TOF are susceptible to cerebral abscess B. The chest X-ray shows oligaemic lung fields C. The second heart sound is soft and single D. TOF is associated with dextroposition of the aorta E. This disorder results in a left to right shunt

Teaching Notes for Question 56 Theme: Tetralogy of Fallot TOF- 6% of congenital heart disease It comprises: -ventricular septal defect -pulmonary stenosis -overiding of the ventricular septum by the aorta -right ventricular hypertrophy * The VSD is usually large and non-restrictive. * Pulmonary stenosis affects the pulmonary artery, the pulmonary valve and the infundibulum. * Severity is related to the degree of right ou tflow tract obstruction. Patients present with: -Cyanosis - usually not present at birth but develops over the first year. -'Hypercyanotic attacks' - due to spasm of the infundibular muscle. During these attacks the infant becomes cyanosed, restless and tachypnoeic. Loss of consciousness may occur. On auscultation, the murmur may decrease in intensity -Squatting - this increases systemic blood pressure, and increases pulmonary blood flow -Clubbing, pan or ejection systolic murmur due to right ventricul ar obstruction, single second heart sound, soft P2 -Failure to thrive -Polycythaemia -CXR findings: * boot-shaped heart (enlarged right ventricle) * right aortic arch -25% * small or concave pulmonary artery * oligaemic lung fields -ECG shows right axis deviation, right atrial and right ventricular hypertrophy -Echocardiogram is diagnostic -cardiac catheterisation -angiography Treatment of hypercyanotic spells: -placing the child in the knee-chest position -morphine -beta -blockers to reduce infundibular spasm -bicarbonate to correct the acidosis Antimicrobial prophylaxis is initiated for prevention of endocarditis. Surgery is usually in two stages: -Blalock shunt - the subclavian artery is joined to the pulmonary artery -Waterston operati on - the ascending aorta is joined to the right pulmonary artery Total repair is undertaken at school age. 57-In terms of the NICE guideline of screening for diabetic nephropathy in Type 2 patients: Options: A. The target HbA1c is about 8%. B. A renal consultation is advised if the serum creatinine rises above 250 µmol/l. C. All patients with confirmed microalbuminuria, if no contraindications, should be on an ACE inhibitor. D. Blood pressure is less important than reducing the degree of protein uria. E. Calculating ratios of albumin and creatinine in the urine is not as useful as the total urine protein level.

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PEDIATRICS EXAMINATION REVIEW

Teaching Notes for Question 57 Theme: Diabetic nephropathy For this nephropathy and the retinopathy guideline, one can check; www.nice.org.uk In fact: * target HbA1c is 6.5-7.5% * BP target 135/75 * albumin:creatinine ratio >2.5 (men) and >3.5 (women) is of higher risk * renal consultation advised if creatinine >150 * ACE inhibition is the drug of choice

58-What can you infer from the Chest X-ray? Options: A. Left pneumothorax B. Left lower lobe collapse C. Left lingula collapse D. Left tension pneumothorax E. Left hilar adenopathy

Teaching Notes for Question 58 Theme: Tension pneumothorax * Left hemithorax greate r volume than right * Air outlines right mediastinal border (i.e. has crept posteriorly) 59-Which of the organs below is considered an immunologically privileged site : Options: A. Skin B. Thymus C. Bone marrow D. Testicle

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PEDIATRICS EXAMINATION REVIEW

E. Pancreas

Teaching Notes for Question 59 Theme: Immune privilege Some tissues fail to elicit an immune response and are termed immunologically privileged sites- brain, cornea, testis, uterus and arguably liver. Several factors may contribute to immune privilege. The blood-brain barrier may block entry of lymphocytes into this organ and the avascularity of the cornea may also limit antigen/lymphocyte interaction. The eye also has high conc entrations of TGF-beta which is a cytokine with immunosuppressive properties. Both the eye and the testis constitutively express Fas ligand. This membrane protein can bind to Fas on infiltrating leukocytes, and can induce apoptotic cell death in the Fas ex pressing cell. Liver transplantation may induce antigen -specific tolerogenic effects. The precise mechanisms are not clear, but have been attributed to the establishment of micro chimerism, the existence of tolerogenic donor immature dendritic cells or th eir precursors within the liver graft. In contrast, skin is highly immunogenic. Until modern HLA typing techniques developed, skin grafting was used as a clinical test before solid organ transplantation to determine the tissue type compatibility between t he donor and recipient.

60-Following a dislocated shoulder in a rugby match that gets out of hand, a patient’s axillary nerve is damaged. What is the likely result? Options: A. The teres major atrophies. B. The rounded contour of the shoulder disappears. C. A loss of sensation may occur in the lateral forearm. D. The patient may lose the ability to adduct the arm. E. The patient may have a wrist drop.

Teaching Notes for Question 60 Theme: Axillary nerve The deltoid atrophies when the axillary nerve (C5, 6) is damaged. Therefore, the rounded contour of the shoulder often disappears. A loss of sensation may occur over the ‘epaulette’ area of the shoulder. 61-Steve, a 10-year-old boy presents with a chronic cough, recurrent lung infections and clinical evidence of obstructive pulmonary disease. His mother reveals that when he was 2 years old he was diagnosed as having ‘malabsorption’, and she has noticed that his s weat tastes excessively salty. All the above are features of this condition EXCEPT: Options: A. 5–10% of cases manifest with meconium ileus at birth B. In 70% of patients, the genetic abnormality is a four -base pair deletion at the locus on chromosome 7 C. Pancreatic abnormalities are present in 80% of patients D. The primary defect is in the transport of chloride ions across epithelial tissue E. Staphylococcus aureus and pseudomonas aeruginosa are two pathogens most commonly isolated from the sputum of patients with this condition

Teaching Notes for Question 61 Theme: Cystic fibrosis

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PEDIATRICS EXAMINATION REVIEW Cystic fibrosis is an autosomal recessive disorder, caused by a mutation in th e gene encoding cystic fibrosis transmembrane conductance regulator (CFTR). The gene product is a cAMP regulated chloride transporter. The CFTR gene is found in region q31 -q32 on the long (q) arm of human chromosome 7. About 70% of mutations observed in CF patients result from deletion of three base pairs in CFTR’s nucleotide sequence. This deletion causes loss of the amino acid phenylalanine located at position 508 in the protein; therefore, this mutation is referred to as deltaF508 CFTR. The disease incidence is about 1/2,300 and the average life expectancy is about 31 years. Abnormalities in epithelial cell chloride and water transport result in viscid secretions in the respiratory tract, pancreas, gastrointestinal tract, sweat glands, and other exocrin e tissues. Patients present with recurrent respiratory infections. Recurrent inflammation and chronic bacterial colonisation with organisms such as staphylococcus aureus, mucoid form of pseudomonas aeroginosa and rare microbes such as burkholderia cepacia result in extensive pulmonary structural damage, bronchiectasis, endstage lung disease and early death. Pancreatic insufficiency occurs in 75% –80% of patients. There is evidence of lipid malabsorption, and failure to thrive. Viscid gastrointestinal secretions may result in meconium ileus at birth and in distal intestinal obstruction syndrome later in life. Increased viscosity of bile with biliary ductule obstruction may ultimately result in cirrhosis. 62-A 26-year-old airhostess is brought to the A&E d epartment following a witnessed grand mal seizure. She has been previously fit and well except for a history of mild intermittent arthralgia affecting both hands. Her only medication is the OCP. She smokes 20 cigarettes per day and drinks 15 units of alc ohol per week. * * * * * On examination she has no focal neurology. There is slight puffiness of her fingers and she has low -grade pyrexia of 37.6C. BP 160/98 mmHg. Fundoscopy reveals silver wiring and AV nipping bilaterally. Urinalysis tests 3+, blood 2+ proteins.

Her blood test results reveal: Hb MCV WCC Platelets CRP ESR Sodium Potassium Urea Creatinine Albumin Tota l protein LFTs BM Clotting screen: PTT APTT VDRL Drug screen of urine Plasma alcohol

9.9 g/dl 83 fl 3.1 x 109/l 117 x 109/l 2 g/l 71 mm in the first hour 134 mmol/l 5.1 mmol/l 10.1 mmol/l 178 mmol/l 30 g/l 80 g/l Normal 7.8 mmol

11 seconds 39 seconds Positive 1:320 Negative Not detected

What is the likely diagnosis? Options:

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64-A boy presents with fever. prolonged APTT and PE are compatible with associated anti-phospholipid syndrome. The medial cord of the brachial plexus. C. The positive VDRL. annular rash in his axilla. Pulmonary embolism D. Lesions of the lateral cord affect the musculocutaneous nerve and the lateral root of the median nerve. and an elevated ESR with normal CRP is indicative of systemic lupus erythematosus (SLE). The ulnar nerve. Infraclavicular injuries may damage the cords. A lesion of this cord will cause to tal paralysis of the small muscles of the hand and have the same effect as a combined ulnar and radial nerve palsy. Kawasaki disease B. Sensory loss would occur at the upper outer arm. The posterior cord supplies the deltoid muscle and all extensors of the arm and fingers. SLE B. The C5 spinal root. He recently visited his GP for a deep IM steroid injectio n. The long thoracic nerve. The probable site of the injection was the: Options: A. B. Infectious mononucleosis C. E.PEDIATRICS EXAMINATION REVIEW A. The vector 184 . Syphilis E. Systemic sclerosis Teaching Notes for Question 62 Theme: SLE The presentation with a fit in the absence of structural or metabolic derangement accompanied with pancytopaenia. Rheumatic fever Teaching Notes for Question 64 Theme: Most likely diagnosis This is a case of Lyme disease . posterior and medial. arthralgia. Scarlet fever D. which his mother says has been present for one week and has increased in size. lateral. Posterior cord of the brachial plexus. Epilepsy C. Lyme disease E. 63-An elderly man presents to casualty with a wrist drop and pain radiating down his right arm. The medial cord supplies the medial branch of the median nerve and the ulnar nerve. arthralgia and headache and a red. This patient has central nervous system (CNS) and renal lupus. This manifest as weakness of forearm flexion and pronation. AVN is a well-recognised complication of steroid treatment. Teaching Notes for Question 63 Theme: Brachial plexus injury The brachial plexus has three cords. Which is the most likely diagnosis? Options: A. D.caused by the spirochaete Borrelia burgdorferi.

Gilles de la Tourette syndrome Teaching Notes for Question 65 Theme: Milkmaid grasp Milkmaid grasp is a feature of Sydenham’s chorea seen in Acute Rheumatic fever. Transmission is by a tick bite or by deposition of B.knee. These include meningitis.PEDIATRICS EXAMINATION REVIEW is the Ixodid species of tick. burgdorferi on the skin in faecal material. heart block. The presence of jaundice would make which one of the following diagnoses more likely? Options: A. cranioneuropathies. heart. malaise. Lyme disease is the most common tick -borne illness in the United States and occurs widely throughout Europe and the former Soviet Union. joints etc. central nervous sys tem. complete heart block and myopericarditis. especially peripheral seventh nerve (Bell palsy)(3%). Toxocariasis D. Diagnosis is confirmed by serology. aseptic meningitis and chronic arthritis. in association with non-specific symptoms such as fever. Generally seen in children. Typhoid B. pronator sign and darting motility of the tongue. encephalitis. * Ocular larva migrans causes a granulomatous reaction in the retina where the larva dies and may be detected on fundoscopy in a child being investigated for strabismus or decreased visual acuity. The spirochaete may disseminate via the lymphatics or the bloodstream to the skin. Late disease presents weeks to months after inoculation. and rarely encephalitis. Disease can localise to heart. but when present includes AV block. Features include VII palsy. Lyme disease E. Malaria causes jaundice because of red cell haemolysis and should always be considered in a child with fever who has been abroad in the past 3 months. uveitis.32 days. eye. wheeze and eosinophilia caused by the immune response to migrating larvae. The annular rash of Lyme disease (erythema chronicum migrans) is usually the first feature. Arthritis is caused by persistence of organisms in the synovium. 66-A 2-year old boy is admitted with fever. The Ixodid tick is harboured by de er in the New Forest. Lyme disease (organism: Borrelia burgdorferi) usually presents with skin lesio ns (erythema chronicum migrans). headache. myalgia. and it tends to enlarge over 1-2 weeks. hepatomegaly. Joint involvement is commonly asymmetrical and usually affects the large joints . arthralgia and malaise. muscle. this chorea has other clinical signs such as the spooning sign. Huntington’s disease D. shoulder and elbow. or Toxocara catis from cats) presents in one of 2 forms: * Visceral larva migrans is a syndrome of fever. Malaria C. headache and constipation. Hemiballismus B. 65-Milkmaid grasp is characteristically seen in which of the following conditions? Options A. 15% of patients present with neurological symptoms and signs. regional lymphadenopathy. Torticollis E. Toxocariasis (organism: the nematode Toxocara canis from dogs. fever. and headache. Typhoid usually presents with fever. Cat scratch fever Teaching Notes for Question 66 Theme: Most likely diagnosis of fever and jaun dice. Treatment is with doxycycline or amoxycillin or IM ceftriaxone if there is neurological involvement. arthralgia. Cardiac disease is relatively uncommon in children. CNS or 185 . Sydenham’s chorea C. Incubation period is 3 .

This is composed of a selection of proteins from pertussis and is said to have fewer side effects than the whole cell pertussis vaccine (which is composed of whole killed organism). On examination. She has antibodies to the core antigen. the acellular pertussis vaccine should be administered the next time. Cat scratch fever (organism: gram negative bacillus Bartonella henselae) presents with (usually cervical) lymphadenopathy and a history of a ca t scratch. He should have all immunisations except pertussis C. In the case of a severe local reaction. Any oral vaccine will be a live vaccine (e.PEDIATRICS EXAMINATION REVIEW joints. Acute hepatitis B infection B. There is no oral pertussis vaccine.5C within 48 hours of pertussis vaccination * Seizures or encephalopathy within 72 hours of pertussis vaccinatio n * Prolonged unresponsiveness or collapse * Anaphylaxis or bronchospasm * Inconsolable screaming for more than 4 hours In the case of a severe generalised reaction. He should receive the whole cell pertussis vaccine (as DTP) as usual at his next immunisation Teaching Notes for Question 67 Theme: Pertussis vaccination This degree of thigh swelling constitutes a severe local reaction (official definition: 'an extensive area of redness which becomes indurated and involves most of the anterolateral surface of the thigh or a major part of the circumference of the upper arm'). or to not yet have formed antibodie s to the core 186 . oral polio (Sabin)). He should receive the oral pertussis vaccine when next immunised E. He should not have any further immunisations B. most of the anterolateral surface of the thigh is red and indurated. A severe generalised reaction is defined as any of the following: * A temperature >39. Chronic hepatitis B infection (low infectivity) D.g. What will you recommend for t his child in the future? Options A. Chronic hepatitis B infection (high infectivity) E. 68-You see a 15-year-old girl in clinic with the following results: * Hepatitis B surface antigen: positive * Antibodies to hepatitis B surface antigen: negative * Hepatitis B 'e' antigen: negative * Antibodies to hepatitis B core antigen: IgG positive * Antibodies to hepatitis B 'e' antigen: positive Which of the following statements best describes her clinical condition? Options: A. He should receive the acellular pertussis vaccine when next immunised D. Previously vaccinated against hepatitis B Teaching Notes for Question 68 Theme: Hepatitis B This girl is HBsAg positive which means she is currently infected with hepatitis B (if she had cleared the infection she would be HBsAg negative and would have antibodies to HBsAg). 67-A two-month-old child had his first DTP injection 3 days previously and his mother has returned because his thigh is swollen. which will always be found in people who have had hepatitis B infection (either currently or in the past). Past hepatitis B infection C. oral typhoid. diphtheria and tetanus ONLY should be given next time. If this was acute hepatitis B infection one would expect her to be IgM positive.

What has he taken? Options: A. Impaired hepatic excret ion of lactate. Causes include tumours sec reting ADH (small cell lung cancer. K. lymphoma). This has relevance if this girl becomes pregnant. The following blood test results are found. prostate. Glue E. However. so dependent oedema is rare. Urine osmolality is >500 mosm/kg E. B. signifies low infectivity. pancreas. Kidney failure. methanol. and paracetamol. HCO3 15. CNS injury. and urate are all lowered. Opiates D. thymus. C. Liver failure. pH 7. ‘e’ -antigen positive (but antibody negative) means high infectivity. He is unconscious and he has a rash around his mouth. The 'e' antigen status determines the infectivity of the carrier. Babies born to mothers with high infectivity require passive and active immunisation (HBIg and vaccine). and solvents (toluene) *glue*. MDMA Teaching Notes for Question 70 Theme: Acidosis Drugs causing high anion gap acidosis include salicylates. 70-A 14 year-old boy is seen in the Ambulance and Emergency Departmen t. and acute intermittent porphyria (AIP).29 . Pco2 4. acetazolamide. babies born to mothers with low infectivity require vaccination alone. Oedema may occur due to fluid overload D. ethylene glycol. creatinine. the fact that she is IgG positive suggests she has chronic infection. drugs. Plasma sodium is usually normal B.PEDIATRICS EXAMINATION REVIEW antigen. Urine is inappropriately concentrated. urea.5. Na 135. serum levels of Na. Another cause of normal anion gap acidosis is renal tubular acidosis. 71-The most important cause of death in infectious mononucleosis is: Options: A. therefore a hyperchloremic acidosis with a normal anion gap. Methanol B. or conversion to acids metabolically can also result in acidosis with high anion gap. 69-In the syndrome of inappropriate ADH secretion (SIADH): Options: A. 187 . Heart failure. antibodies to the ‘e’ antig en (but no ‘e’ antigen detected) as in this case. plasma protein levels are normal. Plasma urate levels are elevated C. Serum globulins are high Teaching Notes for Question 69 Theme: Syndrome of inappropriate ADH secretion (SIADH) SIADH produces inappropriate water re tention despite low plasma osmolality. Ethylene glycol C. lung infections. including Guillain -Barré syndrome. Glue poisoning usually gives rise to a distal (type 1) RTA. K 5. Drugs causing normal anion gap acidosis include amphotericin. Therefore. Cl 110.

and nausea can all occur in acute attacks. Hereditary coproporphyria (HCP) and Variegate porphyria (VP) have skin m anifestations and acute attacks. Teaching Notes for Question 71 Theme: Infectious mononucleosis The spleen is enlarged and sof t in consistency in this disease. Paracetamol. AIP is the only one without skin manifestations. Optic neuritis B. Porphyria rarely presents before puberty and occasionally first presents with an acute acttack triggered by the OCP. Retinitis pigmentosa will cause symptoms of tunnel vision. diarrhoea. Respiratory failure. it may rupture and this is fatal in 25% of cases of infectious mononucleosis. Anterior uveitis C. hirsuitism and blisters as well as acute attacks. “Non -organic” symptoms E. He reports this at a distance of 3 m and also at 6 m. Malabsorption is not a feature of porphyria. Are precipitated by the contraceptive pill E. constipation. Retinitis pigmentosa Teaching Notes for Question 73 Theme: Visual fields Tunnel vision should show an increasing area of vision with distance. Optic neuritis will lead to blurring and ce ntral scotomas. On examination. right arm and shoulder during a game of tennis. he reports having only central vision over an area of approximately 1 m by 1 m. 74-A middle aged lady presents to Casualty with a wrist dro p and pain radiating down her right arm after sustaining an injury to her neck. E. Congenital porphyria (CP) and Erythropoetic Protoporphyria (EPP). aspirin and opiates are safe analgesics in porphyria. 72-Acute attacks of porphyria: Options: A. Anterior uveitis tends to cause pain and blurred vision. Methanol poisoning begins with blurred vision. Which of the following diagnoses b est fits his symptoms? Options: A. A fixed area implies non-organic symptomatology. Methanol poisoning D. The probable site of the injury was the: - 188 . Variegate porphyria is associated with pigmentation. Are associated with malabsorption Teaching Notes for Question 72 Theme: Porphyria Acute attacks do not occur with Porphyria cutanea tarda (PCT).PEDIATRICS EXAMINATION REVIEW D. 73-A 17-yr-old male medical student presents with a sudden onset of tunnel vision. progressing to visual field contraction and sometimes complete blindness. Can be precipitated by paracetamol B. If care is not taken while palpating the spleen. Are a feature of porphyria cutanea tarda D. Can produce hyponatraemia due to Diabetes Insipidus C. Abdominal pain. Splenic rupture.

What is the most likely cause of her hip pain? Options: A. Medial cord of the brachial plexus C.1 mmol/l 10.6°C.1 mmol/l 178 mmol/l 30 g/l 80 g/l normal 7. This arises from the posterior cord of the brachial plexus. she smokes 20/day and drinks 15 units of alcohol per week. T1 spinal root D. There is slight puffiness of the fingers and low -grade pyrexia of 37. Urine analysis tests show 3+ blood. Results: Hb MCV WCC platelets CRP ESR Sodium potassium urea creatinine Albumin total protein LFTs BM Clotting screen: PTT APTT VDRL Drug screen of urine Plasma alcohol 9. Ulnar nerve Teaching Notes for Question 74 Theme: Brachial plexus lesions Wrist drop is indicative of a process affectin g the radial nerve. O2 sats 94% on room air.PEDIATRICS EXAMINATION REVIEW Options: A.8 mmol 11 seconds 39 seconds positive 1:320 negative not detected The patient then develops acute dyspnoea and examinati on reveals decreased air entry at the right base. She responds well to prednisolone and azathioprine. C5 spinal root E. but at an OPD appointment 3 months later is complaining of worsening right hip pain poorly responsive to NSAIDs.9 g/dl 83 fl 3. tachycardia. Avascular necrosis 189 . BP 105/55. 2+ protein. Accidental injection of spinal roots seems unlikely and would not cause a complete palsy. Posterior cord of the brachial plexus B. The ulnar nerve innervates the small muscles of the hand while the medial cord gives rise to the median nerve. 75-A 26-year-old air hostess is brought to A&E following a witnessed grand mal seizure.1 X 109/l 117 X 109/l 2 g/l 71 mm in the first hour 134 mmol/l 5. BP 160/98 mmHg. Her only medication is the OCP . She has been previously fit and well except for a history of mild intermittent arthralgia affecting both hands. X-ray is normal but bone scan shows increased uptake in the hip. Fundoscopy reveals silver wiring and AV nipping bilaterally. On examination she has no focal neurology. Osteoporosis B.

Cytomegalovirus E. I f it is not suppressed then the diagnosis of Cushing’s syndrome is made after taking the urinary cortisol results into account. diarrhoea. however 10 days after admission she develops a fever. * Commonest clinical features of Cushi ng’s include hypertension. headaches and a non -productive cough. Klebsiella pneumoniae C.PEDIATRICS EXAMINATION REVIEW C. High-dose dexamethasone suppression test C. High -dose dexamethasone test is a valuable outpatient screening test. diabetes mellitus and muscle wasting. osteoporosis. Her 24 -hour urinary free cortisol is 450 nmol. Cryptococcus neoformans 190 . central obesity with striae. She is given treatment with prednisolone and cyclosporin. Which is the next investigation to confirm the diagnosis? Options: A. Pneumocystis carinii D. Septic arthritis D. however after 48 hours the fevers continue and a repeat CXR shows left lower lobe consolidation. and ectopic ACTH production is responsible for the remaining 10%. 77-A 26-year-old woman is admitted to hospital for cadaveric renal transplantation. This patient has CNS and renal lupus. She is treated with cefotaxime. arthralgia. a nd an elevated ESR with normal CRP is indicative of SLE. AVN is a well-recognised complication of steroid treatment. Measuring ACTH levels E. Streptococcus pneumoniae B.Complications The presentation with a fit in the absence of structural or metabolic derangement accompanied with pancytopenia. skin fragility. adrenal neoplasms are responsible for around 30% . Blood cultures are taken and a CXR is unremarkable. * Cushing’s disease is the cause of Cushing’s syndrome in around 60% of cases. * Prognosis is poorest in patients where the cause is malignant adrenal neoplastic disease. Options: A. hirsuitism. 76-A 35-year-old woman who complained of easy bruising. Measuring cortisol levels D. CRH stimulation test Teaching Notes for Question 76 Theme: Cushing’s syndrome * 48-hour low-dose dexamethasone suppression test is the investigation of choice to confirm the diagnosis in cases of Cushing’s syndrome in which urinary free cortisol is abnormally high. The positive VDRL. whereas the low dose dexamethasone test is conducted while the patient is admitted in the hospital. Stress fracture E. Trochanteric bursitis Teaching Notes for Question 75 Theme: SLE . Low-dose dexamethasone suppression test B. 0. weight gain and striae is being worked up for Cushing’s syndrome. those with surgically amenable lesions tend to do well.5 mg of dexamethasone is given every 6 hours till 8 doses are completed and suppression results are noticed. The operation is uneventful however she is admitted to the Intensive Therapy unit for 48 hours postoperatively. prolonged APTT and PE is compatible with associated anti -phospholipid syndrome. emotional lability. Examination reveals no focal signs of infection.

iv isolyte – P C. The dehydration may result in hypernatraemia or hyponatraemia and there may be pre -renal renal failure. Diagnosis can be made from stool culture 191 . iv 50% calcium gluconate B. and is used in the treatment of paediatric dehydration. On examination the child is afebrile and dehydrated with a depressed anterior fontanelle. Intravenous (iv) Isolyte P consists of 5% dextrose with Na.)Dyselectrolytaemia 2. they should be considered as a cause of invasive infections. metabolic alkalosis is the classic electrolyte and acid -base imbalance associated with the non -bilious vomiting of pyloric stenosis. hypokalaemic. Two major diagnostic considerations are:1. The intravenous (iv) fluid of choice for administration would be: Options: A. Preexisting immunocompromise is a risk factor C. CMV and C. iv 5% dextrose D. resistance to 3rd generation cephalosporins is common in the noscomial setting. particularly in individuals who had been nursed in renal and high dependency units. 78-A 6 week old male baby presents with a 2 day history of multiple seizures. Ultrasound of the abdomen shows hypertrophic pyloric stenosis. iv Hypertonic saline Teaching Notes for Question 78 Theme: Treatment of electrolyte imbalance This child presents with a history of seizures that are of recent origin and vomiting for 3 weeks. Pontiac fever is a rarer and milder form of the disease E. This raises the possibility of atypical org anisms. a response to Cefotaxime would be expected within 48 hours. Although coliforms are frequently isolated from the sputum of hospitalised patients. He has been vomiting non-billious breast milk for the last 3 weeks. neoformans are very unlikely. sodium bicarbonate or hypertonic saline woul d also not address the basic electrolyte imbalance. He is dehydrated and afebrile with a depres sed anterior fontanelle (AF). K and Cl supplementation.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 77 Theme: Nosocomial pneu monia Although Streptococcus pneumoniae should be considered as one of the most likely causes of postoperative pneumonia in an immunosuppressed patient. however this soon after a transplant P. Hypochloraemic. Strict barrier nursing is essential to contain the infectious agent B. carinii. Obviously serum electrolytes will be measured. 79-Regarding Legionnaire's disease:Options: A. Replacement should be guided by the clinical response and the electrolyte balance. Metabolic alkalosis causes a decrease in ionised calcium because of increased binding of calcium to plasma proteins.)Septicaemia and meningitis A septic screen should be performed and antibiotics commenced. particularly those who may have had many previous courses of antibiotics. iv sodium bicarbonate E. Infusion of calcium gluconate would not correct the metabolic abnormality. Infusion of 5% dextrose. Rifampicin alone is as effective as erythromycin for the treatment D.

affects younger patients. For example. pneumococcal pneumonia and mycoplasma pneumonia.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 79 Theme: Legionnaire's dis ease Legionnaire's disease is usually due to infection with Legionella pneumophila type 1. Thick and thin blood film B. 82-A 15-year-old is undergoing investigation for hirsuitism. epidemic tendency. CXR shows hazy shadowing in the right mid and lower zones. Which of the following drugs is the likely cause : Options: A. high alcohol intake. smoking. Blood cultures E. Cefotaxime B. The least useful test is: Options: A. pre -existing immunocompromise and COPD are risk factors. However cerebellar ataxia. Penicillin C. multiple thick and thin blood films are essential. Rifampicin D. Ciprofloxacin E. a 16 -year-old boy devel ops fever and rigors. Male sex. For Legionnaires' disease. Rifampicin alone increases resistance and is not as effective as 3 weeks of erythromycin. IgM for viral haemorrhagic fever C. CXR and blood cultures are needed to rule out a respira tory infection or systemic sepsis. 80-A 15 year old boy is admitted with pneumonia. Clinical differentiation from the other caus es of community acquired pneumonia is not possible. Chest radiograph D. 81-Seven weeks after returning from rural Ghana. Tetracycline Teaching Notes for Question 80 Theme: Drug induced jaundice (1) The patient was started on rifampicin for suspected Legionella pneumonia. has a shorter duration. 7 weeks is too long. the incubation period is 2–21 days. no association with underlying diseas e and zero mortality. HIV antibody test Teaching Notes for Question 81 Theme: Fever in a returning traveller Malaria is the most likely tropical diagnosis. Investigations show hyponatraemia and mildly deranged LFTs. peripheral neuropathy and poor memory are recognise d neurological complications and are probably mediated by a cerebral toxin (in addition to the effects of hyponatraemia). and patient should be warned that secretions will turn red (particularly tears and urine). Viral haemorrhagic fever should be considered in the differential diagnosis if the patient has returned from an endemic area within the last 21 days. rifampicin should be given with erythromycin as when given alone it tends to increase resistance. Which of the following would not be implicated in the aetiology? Options: 192 . Rifampicin is a hepatic enzyme inducer and can lead to acute jaundice. Therapy is started and 5 days later he becomes acutely jaundiced with red discolouration of the urine. therefore. It is a relatively common cause of community-acquired and nosocomial pneumonia in adults much less common in children. Pontiac fever is a 'flu -like' illness that is more common than full blown Legionnaire's disease. the incidence of diarrhoea is similar in Legionnaire's disease.

OCP. The pupil appears dilated and does not constrict to light. A karyotype of 46XX excludes a diagnosis of Down’s syndrome E. Polycystic ovaries and congenital adrenal hyperplasia are associated with increased androgens and hirsuitism. The nuchal scan together with bHCG and PAP identifies those at high risk of a Down’s syndrome pregnancy in >80% of cases. but there is no need to karyotye the affected child B. She complains of discomfort in the eye and thinks that difficulty with perception of colour was the first problem that she noticed. visual acuity on the left is down to light perception. Phenytoin. Consequently. Women over 35 are at increased risk of having a child with Down’s syndrome but because most children are born to mothers <35 years. spironolactone). reducing free androgens (oestrogen therapy. The triple test consists of measurements of alpha fetoprotein. weight loss). They have one child affected with Down’s syndrome and are considering another pregnancy. D. Treatment is with anti-androgens (cyproterone. Optic neuritis 193 . On examination. Which of the following statements is most accurate? Options: A. B. Moxonidine. 84-A 17 year old student develops a deterioration in vision in her left eye over three days. during a trip to an art gallery. and cosmetic. most children with Down’s syndrome are born to younger mothers. Because of this. CMV retinitis B. It will identify about 65–70% of those at high risk of carrying an infant with Down’s syndrome. Teaching Notes for Question 82 Theme: Hirsuitism Drugs causing hirsuitism include cyclosporin/phenytoin/minoxidil (not moxonidine -a centrally acting anti-hypertensive). some will occur because of a balanced chromosomal translocat ion in one of the parents. 83-A family attends g enetic counselling.PEDIATRICS EXAMINATION REVIEW A. they have a high recurrence risk for other children to be affe cted with Down’s syndrome. What is the most likely diagnosis : Options: A. Both parents should have karyotype analysis. Congenital adrenal hyperplasia. A nuchal scan together with bHCG and PAP (pregnancy associated protein) between 11 and 13 weeks of pregnancy gives an accurate risk of Down’s syndrome in >80% of cases Teaching Notes for Question 83 Theme: Trisomy 21 Although most cases of Down’s syndrome occur because of a complete trisomy of chromosome 21. E. Cushing's syndrome. Most children with Down's syndrome are born to women over the age of 35 years C. Amniocentesis should only be offered to confirm the diagnosis of Down’s syndrome if the family will consider termination of the pregnancy D. all families with a child with Down’s syndrome should have basic chromosomal investigations performed. although does when a torch is shone in the right eye. C. bHCG and unconjugated estriol. Cyclosporin A. The parents in these cases are phenotypically normal and have a complete complement of genetic material but the chromosomal arrangement is different.

Aseptic meningitis is a recognised clinical manifestation of FMF. scalp tenderness. therefore requires appropriate investigation.1 mmol/l no organisms seen on microscopy 48 hour culture of CSF negative What is the diagnosis? Options: A. May be the first manifestation of MS. Anterior iscaemic optic neuropathy D. hypertension and diabetes are risk factors. Adult onset Still's disease C. Central retinal artery occlusion Teaching Notes for Question 84 Theme: Unilateral visual loss Causes of acute Unilateral loss of vision Acute angle glaucoma – other eye is often affected Temporal arteritis – Associated with headache. may be central field defect with colour disturbance a prominent feature. pleuritic pain. Toxoplasmosis in association with immunodeficiency 85-A 29 year old Cypriot man gives a history of recurrent pain and swelling of the left knee. Red spot at macula. Behcet’s syndrome D. ENA and ANCA negative CSF analysis: opening pressure 14cmH2O protein 0. with maximal deficit at the onset and variable resolution.PEDIATRICS EXAMINATION REVIEW C. He is then hospitalised with severe headache. Amaurosis Fugax E. Associated with vascular risk factors as usually embolic. Familial Hibernian fever E.5 mmol/l CRP 39g/l ESR 56 mm in the first hour RF. He also describes recurrent episodes of fever.8g/dl plats 210 X 109/l WCC 4. Optic Neuritis – Painful. Amyloidosis can complicate any condition characterised by a prolonged persistent acute 194 . High ESR. Familial Mediterranean fever B. His blood test results show: Hb 13. May not result in complete visual loss. Amaurosis Fugax – Transient blindness lasting minutes to hours. and rash. Arteriosclerosis. Non Hodgkin’s lymphoma Teaching Notes for Question 85 Theme: Familial Mediterranean fever (aseptic meningitis) Pleuritis can occur in Familial Mediterranean fever(FMF)whereas peritonitis occurs much more commonly. photophobia and neck stiffness. Infectious C auses – CMV. colour vision affected. Usually affects both eyes. Central Retinal Artery Occlusion – Acute. not an infrequent consequence of untreated FMF. He is lucid with a GCS of 15.6 g/l RBC 2 /ml WBC 21/ml (100% lymphocytes) CSF glucose 4. jaw claudication. ANA. May be described as a “curtain coming down”Anterior Iscaemic Optic Neuropathy – Painless.9 X 109/l plasma glucose 6. The splenomegaly and proteinuria is due to the development of amyloidosis.

PEDIATRICS EXAMINATION REVIEW phase response. The Marie -nostrin gene is only found in 85% of patients with FMF. The SAP scan is a quantative investigation for the extent of amyloidosis. Colchicine given prophylactically in FMF is thought to offer some protection against the development of amyloidosis. 86-Which of the following do not tend to make the symptoms of myasthenia gravis worse? Options: A. Prednisolone B. Aminoglycosides C. Pyridostigmine D. Lithium E. Quinidine

Teaching Notes for Question 86 Theme: Myasthenia gravis Although prednisolone is used in the treatment of myasthenia, it may make the symptoms worse, initially. The main drugs to be regarded with caution are: * Aminoglycosides * Procainamide * Quinidine * Lithium * Phenytoin * Penicillamine * Contrast agents.

87-This 15-year-old was cyanosed on exertion. What is the diagnosis? Options: A. Pulmonary thromboembolic disease B. Fallot’s tetralogy

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C. Transposition of great arteries D. Eisenmenger’s syndrome E. Mitral stenosis

Teaching Notes for Question 87 Theme: TOF This is a classical “boot-shaped heart” with an absent pulmonary artery im pression and oligaemic lungs. 88-A patient has hypothyroidism. She develops a painful hand at night. In view of the probable aetiology, which muscle would you look for wasting? Options: A. Lateral two interossei B. Abductor pollicis brevis C. Medial two lumbricals D. Flexor digiti minimi E. Extensor pollicis

Teaching Notes for Question 88 Theme: Carpal tunnel. In the hand, the median nerve supplies the: * lateral 2 lumbricals, * opponens pollicis, * abductor pollicis brevis, and * flexor pollicis brevis - (LOAF). The other muscles of the hand are innervated by the ulnar nerve. 89-In a patient that has been previously diagnosed with Wilson’s disease, which o f the following statements is true : Options: A. Serum cerruloplasmin levels are raised B. When penicillamine is administrated then urinary copper level rise C. Is inherited as an autosomial dominant trait D. May result in retinal abnormalities E. Most common presentation is with a manic depressive illness

Teaching Notes for Question 89 Theme: Wilsons disease Wilson's disease most commonly presents with extra -pyramidal features and dementia. Serum cerruloplasmin is low, Kaiser Fleisher rings appears at the periphery of the iris. Autosomal recessive inheritance, gene is on chromosome 13. 90-Which of the following statement(s) regarding childhood minimal -change nephrotic syndrome is/are true?: Options; A. On immunofluorescent studies, deposition is seen along the glomerular basement membrane B. Hypervolaemia is a common problem C. Albumin is routinely given to children with proteinuria D. Long-term cyclophosphamide therapy is beneficial in the steroid resistant group

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E. Minimal change disease is a histological diagnosis

Teaching Notes for Question 90 Theme: Nephrotic Syndrome * By definition this is “minimal change”; only Electron microscopy changes in the glomerular basement mambrane are seen in the form of increased spaces between the podocytes or foot processes. This makes the glomerulus more permeable and albuminuria occurs. * These children are far more likely to be hypovolaemic. * 4.5% albumin is given if the child is severely hypovolaemic (as resuscitation fluid).20% albumin is given for severe oedema, especially tense ascites, scrotal oedema. * Cyclophosphamide is beneficial but only as a short -term course over 8 weeks. The total cumulative (lifetime) dose of cyclophosphamide must not exceed 180mg/kg so as to avoid the side -effects of gonadal toxicity and late malignancies. * MCNS is a histological diagnosis – the term steroid sensitive NS is used if a clinical diagnosis is made. PROTOCOL FOR MANAGEMENT OF NEPHROTIC SYNDROME: 1.Initial episode: Prednisolone 60 mg/m2/day (max 80 mg/day) x 4 -6 weeks, followed by 40 mg/m2 on alternate days for 4 wks 2.First two relapses: Prednisolone 60mg/m2/day (max 80 mg/day) until remission followed by 40mg/m2 on alternate days for 4 weeks 3.Frequent relapser: Maintenance Prednisolone 0.1 -0.5 mg/kg/alt days for 3-6 months, then slow reduction 4.Relapse on Prednisolone > 0.5 mg/kg/ alternate days Levamisole 2.5 mg/ kg /alternate days 5.Relapse on Prednisolone >0.5 mg /kg/alt days with steroid side effects or >1.0 mg/kg/alt days cyclophosphamide 2-3 mg/kg/day for 8 weeks 6.Post - cyclophosphamide relapses: As 2-3 above or Cyclosporin 5 mg/kg/day for one year Indications for renal biopsy in a child with nephrotic syndrome •Age< 1yr (congenital nephrotic synd) or >16 yrs (adult disease pattern) •Persistent HT •Persistent or macroscopic hematuria •Persistent low C3 or C4 •Impaired renal function unresponsive to correction of fluid balance •Before second line agents are started – ie not steroid sensitive. What are the complications of Nephrotic Syndrome? Infection•due to urinary loss of immunoglobulins •loss of factor b of the alternate complement activation path •loss of transferrin •T cell abnormalities •steroid immunosuppression •Cellulitis (staph), peritonitis(pneumococcal), •Septicaemia (gr negative ),chicken pox, measles Hypovolaemia- abdo pain, oliguria, cold extremities, increased toe core temp gap, poor cap refill, BP may be normal ,high (reactive HT) or low •Raised Hb/Hct, low urinary Na (< 10 mmol/l) Thrombosis and embolism •Hypercoagulability due to increased plasma fibrinogen, factor V & VII b y liver, decreased antithrombin III (lost in urine) + hypovolaemia, thrombocytosis, oedema & reduced mobility, diuretics Hypocalcaemia- due to loss of vitamin D binding protein in urine(also albumin loss - need corrected Ca++ Complications of treatment•· Cushingoid effects, poor growth, cataract - steroid(5% mortality), •· Bone marrow suppression (cyclophasphamide, cyclosporin) 91-A child has had his chromosomes analysed in the course of various investigations and the result is: 46 XY, t (2;5)(q35;p21. 3}.

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Which of the following statements is correct?: Options: A. He has more than 46 chromosomes B. The result shows all his genetic defects C. There is a translocation between the short arm of chromosome 2 and the short arm of chromosome 5 D. He is likely to be infertile E. There is an increased risk of him having a child with difficulties

Teaching Notes for Question 91 Theme: An abnormal karyotype The term karyotype is used to describe an individual’s chromosomal complement. It will accurately describe visible abnormalities in chromosome number and arrangements such as translocations, deletions and duplications. It does not describe an individual's genetic defects. Every individual has two copies of over 40,000 genes and many of these will have mutations that are of no consequence to the individual. The karyotype 46 XY, t (2;5)(q35;p21.3) describes a male with a normal number of chromosomes but a translocation be tween the long arm of chromosome 2 (q) (sub band 35) and the short arm of chromosome 5 (p) (sub band 21.3). An individual with a balanced translocation such as this is likely to be asymptomatic but is at risk of having a child with difficulties. Y chromosome abnormalities are much more likely to result in infertility. 92-In leptospirosis which one of the following is true? Options: A. It is usually transmitted by rat bite B. It is a notifiable disease C. The patient may be jaundiced during the immune phase D. The organism may be isolated from urine during the septic phase E. Ciprofloxacin is the treatment of choice for severe forms

Teaching Notes for Question 92 Theme: Leptospirosis * Leptospirosis is a zoonosis transmitted by contact with water contaminated with rat urine. * Most patients suffer only a mild fever headache and muscle pains and the condition often goes undiagnosed. In a small number of cases t he disease presents as a septic illness 1–2 weeks after exposure. Weil’s disease is the eponym for the most severe presentation with shock and multi-organ failure. * The organism, Leptospira interrogans can be isolated from the blood or CSF during the septicaemic phase and from the urine during the 2nd week of illness. * Treatment options are IV penicillin/amoxicillin for 7 days in severely ill patients or doxycyline for milder cases. 93-A 9 months old baby girl is brought to you with failure to thrive a nd rickets. Her parents tell you that she has always been a very thirsty child, drinking 3 -4 bottles of water through the night, and having soaking wet napping, urine dipstick shows 1+ glucose, and 1+protein. Which of the following features are you likkely to find on examination and investigation. Options: A. Cataract. B. Corneal opacities. C. Hyperkalaemia. D. Metabolic acidosis with a wide anion gap.

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a bone marrow aspirate confirm that he has acute lymphoblastic leukaemia. -Autosomal recessive.Renal syndrome). -Treatment . *Liver. bicarbonate wasting. *Pancreas. Wbc cystine level. Cystinosis.5. but there are small blasts in the blood film. *Eyes. -All tissues in body involved. Teaching Notes for Question 93 Theme: Renal tubular acidosis. his full blood count is normal . -Once plasma bicarbonate falls low enough urine PH < 5. NAG. -Lysosomal storage disorder . -Hereditary fructosaemia.unable to transport cystine out of lysosome.RBP). Normal value = 8-12 Biochemical features: *Renal glycosuria. 94-A 5 years old presents to casuality with a 2 months history of inguinal lymphadenopathy.esp amphetericin. nephrocalcionosis. PH typically > 6. slit lamp.diabetes. -Can excrete acid in distal tubulee. -Heavy metal poisoning. -Tyrosinaemia. don't get hypercalciuria or nephrocalcinosis. K wasting and tubular proteinuria. -Bicarbonate wasting due t o low threshold. Fanconi syndrome Causes. -Wilson’s disease.corneal deposits. reduced phosphate resorption. Referre him for a bone marrow transplantation.Total numbers of anions. -Low’s syndrome (Oculo -Cerebral. Nephrocalcinosis. Increase cystine -transport out of lysosome. -bicarbonate wasting . Fanconi syndrome. -Idiopathic. Anion gap = Total number of cations in blood . -Rx:small amount NaHco3 Hyperkalemic (TypeIV). This is proximal renal tubular acidosis . -Galactosaemia.impaired function. -Cystinosis. -Diagnosis – clinical suspicion.generalised aminoaciduria . *Proximal (Type II). *Distal (classical type). (B2 microglobulins. -Impaired h+ secretion in distal tubule can not produce acid urine. Phosphocyteamic. 199 .PEDIATRICS EXAMINATION REVIEW E. C. B. -Generalized aminoaciduria (Tubular leak of all low molecular weight proteins). hypercalciuria. -Lwo Po4 bsorption -Hypophosphataemic rickets. Do a lymph node biopsy to exclude concomitant lymphoma. -Antenatal diagnosis available.renal tubular acidosis -Potassium wasting with hypokalaemia. -Rx: large amounts of Nahco3 needed. Renal tubular acidosis. -Pseudohypoaldosteronism. -Aldosteron deficiency. *Kidney-proximal renal tubular acidosis. -Large salt & water loss -polyuria +polydipsia. = (Na+ K ) – (Cl +Hco3).esp 1 fosphamide. Which is the most appropriate initial course of action ? Options: A.large group. can delay onset of renal failure. -Renal glycosuria. -Drugs. -Chemotherapy. Commonce chemotherapy straight away.

atelet antibodies form. lasts for 3 years in boys & 2 years in girls and involves blocks of maintenance chemotherapy and intensive chemotherapy. Long-term heparin therapy may be associated with osteoporosis. hypodiploidy and MLL gene rearrangements. Osteoporosis. Discharge him and review in 1 week time as an out patient. Although the main side effect of anticoagulation therapy is excessive bleeding. may be associated with skin necrosis. Poor prognostic factors include: Age (<1. more unusual side effect exist. Skin necrosis.PEDIATRICS EXAMINATION REVIEW D. Heparin may be associated with both thrombocytopaenia and thrombosis as part of the disorder known as heparin -indused thrombocytopaenia (HITT). especially induction therapy in patients with inherited protein C or S deficiency. Treatment involves stopping heparin and anticoagulating with hirudin or danaparoids. Peripheral neuropathy. and Hb -electrophoresis shows an HbA band only with HbA2 quantita tion of 4. This is an acquired immune disorder in which heparin-dependant anti-p.Which of the following is not a recognised complication of anticoagulation thera py? Options: A. B. Sickle cell trait. Haematologically normal. Teaching Notes for Question 95 Theme: Anticoagulation. Her partner has a microcytic anaemia. Deep venous thrombosis. all mostly given as outpatients . >10).8%. C. B-thalassaemia trait. Tumoour lysis is common on induction of remossion and best prevented with IV fluids & allopurinol. Sickle-B-thalassaemia. Teaching Notes for Question 94 Theme: Acute lymphoblastic leukaemia Acute lymphoblastic leukaemia is commoner in children. Treatment. Thrombocytopaenia. C. E. Start IV fluids & allopurinol. male sex. E. B. 95. The Hb -electrophoresis shows HbA 55% and Hbs 45%. 200 . E. D. Which diagnosis is impossible in the baby? Options: A. Teaching Notes for Question 96 Theme: Haemoglobinopathy. Warfarin. leading to sever thrombocytopaenia and occasionally catastrophic thrombosis. D. Sickle cell diseas(HbSS). it generally presents with non specific symptoms but it is not uncommon to find lymphadenopathy on presentation . 96-The mother of a baby in the postnatal ward has an antenatal screening test for sickle haemoglobin. This is positive deposite a normal Hb & MCV.

*B-thalassaemia trait . C. Teaching Notes for Question 98 Theme: IgA nephropathy. The baby may therefore have : *Normal Hb. low grade fever and has some cervical lymphadenopathy. D. *Sickle cell trait.PEDIATRICS EXAMINATION REVIEW The mother probably has a sickle cell trait . C. At 15 years age. B. on consulting his GP 3 days after the onset of this illness. and immediately follows a viral infection or may have no clear precipitating factor. If a child has tried many treatments over a number of years the prognosis is poor. IgA nephropathy (Berger’s disease) typically affects young adult males. 10% develop renal failure.42g/l (0.20)C4= 0.4 urea=10.1 . Proteinuria rather than haematuria is more common in PSGN. B.CH50= 87% (50-110) Urinalysis: blood 3+. protein +. There is a 30% chance that his father was a bed-wetter. 98-A 18 years old student becomes un well with a sore throat. *Children with enuresis wet the bed deliberately. 97-A 9 years old male has always wet the bed. What is the most likely diagnosis? Options: A. Teaching Notes for Question 97 Theme: Enuresis.31g/l (0. investigations reveals: FBC normal .the father has no evidence of Hbs & has a laboratory & Hb-electrophoretic profile compatible with B 0thalassaemia trait. E.20-0. E. there is a 1% chance that he wi ll still bed-wet. wh ich one of the following statement(s) regarding enuresis is not true? Options: A. Henoch-Schonlein purpura. *Sickle-b-thalassaemia. Runs in families. few red cell casts. post-streptococcal glomerulonephritis. Some of the myths regarding enuresis are: *Medications are required in all cases.55 1. he has no daytime enuresis. It is not possible for the baby to have sickle cell disease (HbSS).C3= 1. examination is otherwise unremarkable. Primary nocturnal enuresis. *The bladders of children with enuresis are smaller than other children. post -streptococcal GN normally follows a URTI after 2-3 weeks % is associated with a low C3 &coplement consumption where as C3 is normal/high in IgA nephropathy. Drinking tea causes bed-wetting D.Na=137 K=4. Ig A nephropathy. Interstitial nephrosis.50). Wegener’s granulomatosis. There is no history of antibiotics or NSAIDs for interstitial nephritis and no history of arthralgia or purpura f or HSP 201 .creatinin =141 . Prognosis is good.

Stomach distension increases colonic motility. in addition to old age. Mucous. K and bicarbonate are absorbed. In arround 3% of cases death occurs . C. 202 . presence of postural hypotension. and secretion of k. on examination he has flacid weakness predominantly in the legs and absent reflexes. Teaching Notes for Question 99 Theme: Prognostic factors in Guillain Barre syndrome. Denervation (rather thyan demyelination) show on nerve conduc tion studies implies a poor prognosis as do the remaining stems. D. History of diarrhea in the preceding few weeks. C. Na and Cl are actively secreted. Sever weakness.up to 10% of cases have sever residual disability. Markedly reduced FVC. The diagnosis in Guillain Barre syndrome. CSF examination shows markedly raised protein & a mild lymphocytosis. It is the site of most vitamin C absorption. Increased parasympathetic activity leads to decreased co lonic motility. Parasympathetic activity.PEDIATRICS EXAMINATION REVIEW 99-An adolescent male is admitted with progressive limb weakness. emotional factors and cholinergic agents increase colonic motility. hco3 and mucous. Evidence demyelination on nerve conduction studies. E. D. Vitamin C is water -soluble (acscorbic acid) and most absorption is proximal to the colon. distension (gastrocolic reflex). E. Teaching Notes for Question 100 Theme: Colonic physiology. Which of the following does not imply a worse prognosis? Options: A. B. B. Colonic functions include active absorption of Na and cl. 100-Only one of the following is correct regarding physiology of the colon: Options: A.

PEDIATRICS EXAMINATION REVIEW Questions (PART 3) SELECT THE MOST APPROPRIOT DIAGNOSIS 203 .

On investigation there is lymphocytosis with atypical lymphocytes. 3-Options : A. A 5 year old girl presents with conjunctivitis. CT scan E. fever and coryza 12 days after exposure to a sick sibling. Varicella zoster K. Mumps J. and has painful swelling of both parotid glands. Each option may be used once. Marrow biopsy C. A 8 year old boy presents with malaise. 2. Osteomalacia B. 2-Options : A. 4. cervical lymphadenopathy and erythema of palms and soles. juxtaarticular osteoporosis and bony erosions. sore throat. Kawasaki’s disease E. A 6 year old girl presents with fever and a slapped cheek erythematous eruption on her cheeks. INR F. Paget’s disease Instructions: Choose the most appropriate disease for the radiological description. 1. malaise. anorexia. Tuberculosis G. fever. Mixed osteoblastic and osteolytic lesions in the left tibia. Osteoarthritis F. Avascular necrosis D. Narrowing of joint space.PEDIATRICS EXAMINATION REVIEW 1-Options : A. Full blood count B. Measles Instructions: For each of the patients described below choose the single most likely option from the above list. 5. more than once or not at all. Typhus F. Rubella L. He has enlarged cervical lymph nodes. A 6 year old boy presents with fever. Infectious mononucleosis H. 4. On examination she has blue white punctate lesions on her buccal mucosa. 3. Pneumococcal infection D. 2. There is also cortical thickening. Bamboo spine 3. Erythema infectiosum I. Joint space narrowing. Rheumatoid arthritis E. Thyroid function tests 204 . EEG D. Ankylosing spondylitis C. Haemophilus influenza epiglottitis C. Streptococcal throat infection B. A 16 year old boy presents with fever. 1. marginal osteophytes and subchondral cysts.

Intussusception Instructions: Please match the most likely diagnosis with the scenario described below 1. A baby girl born at 26 weeks is transferred to NICU on day 3 with abdominal distention. with absence of secondary sexual characters. Breast ultrasound Instructions: Drug side effects-investigations. microcephaly. Malrotation F. Patau’s syndrome D. cubitus valgus. Malrotation with volvulus G. more than once or not at all. 37. Cystic fibrosis F. anorexia. jaundice. A 5-year-old child is brought to the hospital with complaints of repeated chest infections and GI problems since birth. 1. His sweat chloride levels are diagnostic. 5-Options A. Patient on carbamazepine feeling unwell. Each option maybe used once. Drug serum levels K. anosmia. 5. Duodenal atresia B. 1. Oesophageal atresia D. Necrotising enterocolitis E. with delayed puberty. tremor. Jejunal atresia C. 3. 2. Silver Russell syn drome Instructions: From the given list of options. Each answer may be used once. drowsiness 3. Hirschprung's disease H. Kleinfelter's syndrome G. Liver biopsy I. with sore troat. A young mother brings her child to the paediatric OPD as she noticed h e has absence of skin on his scalp area. Kallmann’s syndrome H. large tongue.2 °C 4. and a VSD. choose the single best answer for the given set of questions. Patient on Sodium Valproate. cleft lip and palate and mostly normal mental development. Prolactine levels J. A tall adult male. Male patient on phenothiazines comes with discharge from his breast 4-Options A. Cri-du-chat syndrome E. O/E. with diplopia. Liver function tests H. and temp. Turner's syndrome C. Down's syndrome B. Patient on phenytoin. A mother brings her 2 year old with respiratory infection to the OPD. blood in the stools and increasing ventilatory requirements 205 .micrognathia. more than once or not used at all.PEDIATRICS EXAMINATION REVIEW G. and low mood 2. A young female. with vomiting. retarded physically and mentally. O/E he has simian crease. heart defe cts and mild mental retardation. 4. umbilical hernia and a pansystolic murmur of VSD are present. give the most appropriate investigation for each scenario.

6 x 109/L White cell count Neutrophils Lymphocytes Monocytes Eosinophils Basophils Platelets Reticulocyte count Prothrombin time Fibrinogen D-Dimer screen Serum sodium Serum potassium Serum urea Serum creatinine Lactate dehydrogenase 25. A baby boy born at 32 weeks is transferred to NICU on day 1 with bilious vomitting. On auscultation there is a continuous murmur. 4. 6-Options A. A friend.8 x 109/L 22. some blood in the stool and a silent abdomen.8 x 109/L 0. 7-An 18-year-old man was admitted to casualty at 3 am having collapsed at a nightclub.3 mmol/L 17. Atrial septal defect B. 4. Coarctation of aorta E. Investigations revealed: Haemoglobin 8. On examination there is an absence of femoral pulses with a mid -systolic murmur heard over the back. On examination. He has bilious aspirates.0 x 109/L 0. told the paramedics that his friend had been well earlier in the evening and that he usually did not drink much alcohol or take any drugs.1 x 109/L 32 x 109/L 12% 32 s 0. Patent ductus arteriosus D. 3. he has features of Trisomy 21.5 mmol/L 200 mmol/L 540 IU/L Activated partial thromboplastin time 90 s The blood film showed red cell fragmentation with polychromasia. leg fatigue and claudication.0 mg/L 140 mmol/L 6. Ebstein anomaly Instructions: Match the correct physical sign/presenting symptoms with the lesion of congenital heart disease:1. A 3 year old presents with breathlessness and pedal oedema.PEDIATRICS EXAMINATION REVIEW 2. A 7 year old presents with breathlessness. 5. On auscultation there is a sharp holosystolic murmur heard along the left sternal border. 5. A 18 year old presents for a health checkup with no symptoms. A baby boy born at term is transferred to NICU on day 2 with abdominal distension and failure to pass meconium. An 18 year old presents with palpitations. On examina tion there is a widely split fixed 2nd heart sound. A baby girl born at 36 weeks is transferred to NICU on day 1 with cyano sis and respiratory distress after attempted feeding 3. toxic granulation of neutrophils and platelet anisocytosis. 2.5 x 109/L 2. A 17 year old presents with headache. who h ad accompanied him in the ambulance but subsequently left A and E before speaking to a doctor. What is the diagnosis? 206 .3 g/L 3. A baby boy born at term is transferred to NICU on day 10 with sudden collapse. Ventricular septal defect C. On auscultation there is a quintuple cardiac cadence.4 x 109/L 0.

co-trimoxazole and aciclovir. 1. which she notes on the toilet paper. Autoimmune thrombocytopaenia C. with a trace o f blood on his anus. and complains of central abdominal pain. No organs were palpable. A 1 year old child has been noted to have bouts of screaming where he is doubled -up in agony from abdominal pain with a 60 minute pain -free period in between. and an abdominal mass. Nephroblastoma I. you note a redcurrant jelly stool. Autoimmune haemolysis B. On examination. more than once or not at all.4 g/dL Mean corpuscular volume (MCV) White cell count Neutrophils Lymphocytes Monocytes 90 fL 3. A 13 year old girl presents with a complaint of abdominal pain that she has experienced for the past two weeks. which has worsened acutely today. Disseminated intravascular coagulation D. penicillin V. A per rectum examination is too tender to perform and an anal fissure is noted at 6 o'clock. Bowel sounds were active. Six weeks later he presented with a rash. 4. Appendicitis E. headache and nausea. and on palpation the liver is non -tender. His abdomen was soft apart from mild tenderness in the right upper quadrant. a sore throat. His pulse was 104 beats per minute (bpm) regular. 9-A 18-years-old adolescent had a bone marrow transplant from his sister for treatment of acute myeloid leukemia. Mesenteric adenitis C. Strangulated inguinal hernia F.PEDIATRICS EXAMINATION REVIEW Options : A. Today he has vomited twice. Intussusception B. His med ications included cyclosporin. When you ask to see his nappy. Drug-induced haemolysis E. is not tolerating fluids. A 2 year old girl is referred urg ently to clinic having presented to her general practitioner with haematuria on two occassions. 2. A 4 year ol d boy is brought into hospital by his mother.0 x 109/L 0. For the past 2 weeks he has had a cold. green diarrhoea. a diffuse macular rash was visible. On percussion of the liver there is no dullness. Heart sounds were unremarkable and his chest was clear. poor appetite and chronic watery. Hypertrophic pyloric stenosis G. 3. Constipation H. Neurological examination was normal. Her abdomen is soft on palpation and on auscultation there are normal bowel sounds.65 x 109/L 1. History reveals a b right red loss of blood per rectum with defaecation. There is a firm mass with a poorly defined smooth lower border in the left upper quadrant of her abdomen. Each option may be used once.8 x 109/L 207 . Meckel's diverticulum D. mainly affecting the palms of his hands and the soles of his feet.7 x 109/L 1. Riedel's lobe of liver Instructions: Choose the single most likely diagnosis from the options listed above for each of the clinical scenarios described below. Investigations re vealed: Haemoglobin 12. general malaise. Thrombotic thrombocytopenic purpura Choose 1 8-Options A. Blood press ure 112/74 mmHg. She is now asymptomatic.

The GP reported that she has lost about 2 stones over the past 2 months and that although she was preoccupied by preparing food. ECHO M.PEDIATRICS EXAMINATION REVIEW Eosinophils Basophils Platelets Serum albumin Serum total bilirubin Serum alanine transferase Serum alkaline phosphatase Serum gamma glutamyl transferase What is the diagnosis? Options: 0. MRI D.05 x 109/L 0. Absence of depressive features F. Pelvic spring test G. Loss of over 15% of body weight B. Clostridium difficile infection C. Rheumatoid factor. Spinal X-ray and HLA profile B. The mother also informed the GP that despite losing weight her daughter was spending long time every day undertaking heavy exercises. Joint aspiration and microscopy I. Amenorrhea G. 1. she did not seem to be eating well. Which of the following supports a diagnosis of anorexia nervosa in this patient: Options: A.1 x 109/L 147 x 109/L 36 g/L 64 µmol/L 90 IU/L 135 IU/L 40 IU/L Choose 1 A. joint pains and a rash. Sigmoidoscopy. Bone scan C. joint X-ray J. Acute graft versus host disease B. Cytomegalovirus infection D. Binging on fatty food C. Her mother reported that she has been hording food in her bedroom. swab and blood cultures K. Each option may be used once. organomegaly. Transfusion-associated graft versus host disease 10-A 14 year old girl was referred to the local child psychiatrist by her GP who was concerned about her weight loss. joint X-ray L. Increased levels of cortisol E. more than once or not at all. Anti dsDNA and complement E. He also has a 4/6 murmur. 2. biopsy and enema H. Joint aspiration. Choose 5 208 . A 5-year-old Asian refugee child develops high fevers. Use of diuretics 11-Options A. A 45-year-old man who was on a fast developed acute pain in his toe. Side-effect of immunosuppression E. Clinical history alone. Increased levels of growth hormone D. Thomas test F. Neurophysiology Instructions: For each of the patients described below choose the single most likely option from the above list of options.

Pityriasis rosea D. 4. He has some bluish white spots on his buccal mucosa. Meningitis F. Impetigo L. Intrinsic defects in the pattern of development 4. Destruction of normally formed tissue 5. Chemical agent with a malign influence on development 2. Psoriasis E. erythema of the oral cavity.PEDIATRICS EXAMINATION REVIEW 3. He also has a rash which does not blanch on pressure. Measles G. painful and stiff hands and feet and the symptoms are worse in the mornings. A non-immunised child of 5 years presents with catarrh. 5. 12-Options A. and palms. A 3-year-old boy presents with oedema. A 45-year-old man presents with swollen. 13-Options : A. Varicella B. 2. 4. Kawasaki’s disease H. 1. Teratogen F. Malformation D. congested conjunctivae. Dysplasia E. Ebstein-Barr virus J. anorexia. Scabies C. A 45-year-old woman presents with wrist pain. and splenomegaly. It does not respond to antibiotics. which is worse at night and relieved by dangling the hand over the edge of the bed and shaking it. Roseola infantum K. A 6-month-old boy presents with a generalised rash after a bout of fever. fever. palatal petechiae. Abnormal organisation of cells in tissue 14-Options : 209 . conjunctivitis. lymphadenopathy. A 17-year-old African male presents with sore throat. Erythema toxicum Instructions: For each of the patients described below choose the single most likely option from the above list of options: Each option may be used once. Dystocia Instructions: Match the above options with the be st descriptive statement from the choices given below:1. lips. The wound is treated but 2 days later he develops pain in his knee. 5. A man has a bike accident and injures his leg. There is a small open wound. more than once or not at all. fever. Herpes simplex virus I. 3. Deformation B. Disruption C. A mother rings A&E saying that her 3 -year-old boy has fever for 1 day and is drowsy. Abnormal development of a structure caused by extrinsic forces 3. malaise.

sacro -ileitis and rash. Rubella J. Staphylococcus D. depression. A veterinarian presents with fever. Hepatitis N. A 15-year-old patient presents with a rash and suboccipital ly mphadenopathy. anorexia. He is found to have myocarditis. Herpes virus B. hepatosplenomega ly. Her bilirubin is raised. A 35-year-old lady presents with myalgia. Coryza L. He was not immunised as a child. more than once or not at all. Streptococcus C. Meningitis M. malaise. 210 . arthritis. She also has abdominal pain. A man who is on steroids for rheumatoid arthritis for a long period of time now presents with white patches in his mouth.PEDIATRICS EXAMINATION REVIEW A. 3. Poliomyelitis G. Candidiasis H. Diphtheria K. Brucellosis E. His mother gives a history of tonsillitis. vomiting. 5. Tuberculosis F. 4. nausea. Influenza Instructions: For each of the patients described below choose the single most likely option from the above list of options: (Each option may be used once. 2. A 4-year-old boy presents to A&E with shock. and fever.) 1. Infectious mononucleosis I.

PEDIATRICS EXAMINATION REVIEW Answer &Teaching Notes for Questions (PART 3) 211 .

Varicella zoster K. Joint space narrowing. A 8 year old boy presents with malaise. A 6 year old girl presents with fever and a slapped che ek erythematous eruption on her cheeks. Streptococcal throat infection B. 1. 4. Ankylosing spondylitis C. Erythema infectiosum I. Rheumatoid arthritis E. Haemophilus influenza epiglottitis C. 212 . A 5 year old girl presents with conjunctivitis. Tuberculosis G. It is usually monoostotic (affects one bone). A 16 year old boy presents with fever. 2. Avascular necrosis D. sore throat. more than once or not at all. anorex ia. Mumps J. Bamboo spine 3. Pneumococcal infection D. Paget’s disease Instructions: Choose the most appropriate disease for the radiological description.PEDIATRICS EXAMINATION REVIEW 1-Options : A. Teaching Notes for Question 1 Theme: Radiographic abnormalities in rheumatological disease Rheumatoid arthritis and osteoarthritis features are as what given in the question. fever. juxtaarticular osteoporosis and bony erosions. Ankylosing spondylitis affects the spine in young adults and causes restricted spinal mobility. and has painful swelling of both parotid glands. 3. Each option may be used once. Measles Instructions: For each of the patients described below choose the single most likely option from the above list. 4. marginal osteophytes and subchondral cysts. Rubella L. Infectious mononucleosis H. Kawasaki’s disease E. There is also cortical thickening. Osteomalacia B. 2. Most important biochemical abnormality is elevated alkaline phosphatase and normal calcium levels. He has enlarged cervical lymph nodes. Paget’s disease is due to a disorder of bone remodelling. 2-Options : A. Osteoarthritis F. Mixed osteoblastic and osteolytic lesions in the left tibia. 1. malaise. Narrowing of joint space. In late stages X-ray shows bamboo spine where vertebrae are fused. Typhus F. fever and coryza 12 days after exposure to a sick sibling. On investigation there is lymphocytosis with atypical lymphocytes. On examination she has blue white punctate lesions on her buccal mucosa.

PEDIATRICS EXAMINATION REVIEW 5.laryngotracheobronchitis. polymorphous rash and cervi cal lymphadenopathy. The pyrexial phase of the illness lasts up to 10 days and is usually accompanied by intense irritability and fractiousness.21 days. It is caused by parvovirus B19 a small single -strand DNA virus. lymphadenopathy. hepatitis and haemolytic anaemia. 213 .doh. meningitis.otitis media -CNS .postinfectious encephalomyelitis. There should be no delay in giving IVIG once the diagnosis (which is a clinical one) is made. thalassaemia major.ear . seizures. Q3.html.uk/mmr/index. Measles infection presents with catarrh. oral changes -(swollen red lips. This is a case of measles caused by an RNA paramyxovirus spread by droplets. This is a case of mumps caused by a single stranded RNA paramyxovirus. Complications include -arthritis. arthritis. This is a case of Kawasaki disease Kawasaki disease is a vasculitis of unknown aetiology which generally occurs in those under 9 y rs of age. nausea. bronchitis. erythema of the palms and soles (which desquamate in the second or third week of illness). A maculopapular rash typically first appears behind the ears and spreads to involve the face and then the trunk and limbs. palatal petechiae. strawberry tongue). . sore throat. Mortality is around 1% for Kawasaki disease due to acquired cardiovascular disease.this number increases to 80% of patients if there has been previous treatment with amoxicillin or ampicillin. malaise. This is a case of infectious mononucleosis caused by Epstein Barr virus . giant celll pneumonia . splenomegaly. bilateral non -exudative conjunctivitis. Q1. anorexia.gov. This is a case of erythema infectiosum also known as slapped cheek syndrome. malaise. Q4. it should be given within 10 days of the start of the illness. Incubation period is 14 . often followed by an erythematous maculopapular rash on the trunk and legs. Teaching Notes for Question 2 Theme: Diagnosis of childhood illness. Diagnostic criteria include fever. cervical lymphadenopathy and erythema of palms and soles. Q2. These include peric arditis.subacute sclerosing panencephalitis (SSPE) is a rare complication caused by latent measl es virus infection which on reactivation 5 -10 yrs later results in a degenerative CNS disorder. Resolution of the rash revea ls a lacy reticular pattern on the limbs.seen in children with conditions which decrease red cell survival such as sickle cell anaemia. Common symptoms include a mild prodromal illness consisting of fever. Thrombocytosis is a late finding. a bright red rash appears on the cheeks. These are CD8+ T-lymphocytes which are produced in response to EBV infected B -cells. and the smear will show atypical lymphocytes.a virus of the herpes group which infects B-lymphocytes. other h aemolytic anaemias. They are seen early in the illness. The patient is infective 7 days before and 9 days after parotid swelling starts. and rhinorrhea. The infection presents with malaise. focal neurological signs and coma. A 6 year old boy presents with fever. Those wishing to update themselves on the current debate about measles vaccine and autism should read the section and refer to the journals about measles vacccine at web address http://www. A mild erythematous maculopapular rash occurs in 3-15% of patients . myalgia.typically asymmetrical and transient -aplastic crises . It presents usually in young adults with fever. This severe complication usually manifests 7 -14 days after initial presentation with drowsiness. Complications are orchitis. Mortality is 10% and 15% of patients who recover will have residual neurological deficit. Peripheral blood count reveals lymphocytosis. fever and painful swelling of the parotids. headache. myocardititis and coronary artery aneurysm. Incubation period is 8 to 14 days. Q5. Complications are due to infection by the measles virus itself -respiratory. Secondary bacterial infection is an important complication of measles infection particularly in malnourished children. pancreatitis and myocarditis. On day 5 -7 after symptom onset. Koplik spots are pathognomonic 'grain of salt like' spots on the buccal mucosa located adjacent to the posterior molar. Cardiovascular complications occur in the subacute phase of illness (+/_ day 10 to day 30). conjunctivitis and fever.

(I)Neuroleptics by blocking dopamine D2 receptors may give extrapyramidal effects and hyperprolactinemia. Marrow biopsy C. Liver function tests H. Each option maybe used once. CT scan E. (J) Phenytoin levels vary in individuals. His sweat chloride levels are diagnostic. and low mood 2. Q2. Breast ultrasound Instructions: Drug side effects-investigations. give the most appropriate investigation for each scenario. but may occur in any age. A young mother brings her child to the paediatric OPD as she noticed he has absence of skin on his scalp area. A tall adult male. Kallmann’s syndrome H. Kleinfelter's syndrome G. with sore troat. Q4. A 5-year-old child is brought to the hospital with complaints of repeated chest infections and GI problems since birth. 3. drowsiness 3. with vomiting. 2. EEG D. (G) Liver failure is more common in children < 3 years old . anorexia. Patient on phenytoin. more than once or not at all.micrognathia. cleft lip and palate 214 . LFT should be monitored in first 6 months of therapy Q3. with absence of secondary sexual characters. 4-Options A. INR F. Cri-du-chat syndrome E. tremor. Patient on Sodium Valproate. 1. choose the single best answer for the given set of questions. anosmia. microcephaly. with diplopia. Liver biopsy I. Patient on carbamazepine feeling unwell. and temp. umbilical hernia and a pansystolic murmur of VSD are present.PEDIATRICS EXAMINATION REVIEW 3-Options : A. Prolactine levels J. jaundice. more than once or not used at all. (A) Blood dyscrasia with low WBC and frequent infections is a severe side effect. Thyroid function tests G. Down's syndrome B. Male patient on phenothiazines comes with discharge from his breast Teaching Notes for Question 3 Theme: Drug(s) Q1. O/E. 1.2 °C 4. Cystic fibrosis F. plasma concentration monitoring is advised on starti ng the treatment. Patau’s syndrome D. Drug serum levels K. 37. Full blood count B. Silver Russell syndrome Instructions: From the given list of options. Turner's syndrome C. Each answer may be used once.

PEDIATRICS EXAMINATION REVIEW and mostly normal mental development. On examination. Intussusception Instructions: Please match the most likely diagnosis with the scenario described below 1. Malrotation with volvulus G. he has features of Trisomy 21. Children with 'short segment Hirschsprung's disease' can present later in life. choking. Cystic fibrosis occurs due to abnormality in long arm of chromosome 7. A baby boy born at 32 weeks is transferred to NICU on day 1 with bilious vomitting. coughing with episodes of cyanosis exacerbated by feeding. Turner’s syndrome is 45 XO karyotype. Enterocolitis is a severe complication and presents with diarrhoea. 4. 5-Options A. Necrotising enterocolitis E. Rectal biopsy confirms the diagnosis. O/E he has simian crease. some blood in the stool and a silent abdomen. Down’s syndrome is trisomy 21. Oesophageal atresia D. with a history of chronic constipation since birth and poor weight gain. bilious vomiting. Hirschprung's disease is caused by the absence of ganglion cells in the myenteric plexuses of the distal bowel. Kallman’s syndrome is characterised with the features given in the question. retarded physically and mentally. and a VSD. A baby girl born at 36 weeks is transferred to NICU on day 1 with cyanosis and respiratory distress after attempted feeding 3. gross abdominal distension and ci rculatory collapse. cubitus valgus. large tongue. A baby girl born at 26 weeks is transferred to NICU on day 3 with abdominal distention.'long segment Hirschsprung's disease'. 5. Patau’s syndrome is trisomy 13. A baby boy born at term is transferred to NICU on day 10 with sudden collapse. abdominal distension. blood in the stools and increasing ventilatory requirements 2. Duodenal atresia B. 4. Barium enema shows a narrowed distal colon with proximal dilation. Oesophageal atresia has an incidence of about 1 in 2500 live births. There is a high incidence of aspiration pneumonia. Teaching Notes for Question 5 Theme: Neonatal intestinal obstruction 1. There is often polyhydramnios in pregnancy. with delayed puberty. 5. or may extend above the sigmoid . poor feeding and failure to thrive. There is usually an associated tracheo-oesophageal fistula (90%). and there is an association with Down's syndrome (5 -15%) and cardiac malformation (2 -5%). He has bilious aspirates. 5. Teaching Notes for Question 4 Theme: Chromosomal disorders 1. 2. A second enema 2 4 hrs after the first shows retention of contrast and may reveal the transition zone between normal bowel and the distal aganglionic intestine. 2. In 'short segment Hirschsprung's disease'. A mother brings her 2 year old with respiratory infection to the OPD. The child prese nts with frothing from the mouth. 3. Jejunal atresia C. 215 . heart defects and mild mental retardation. A baby boy born at term is transferred to NICU on day 2 with abdominal distension and failure to pass meconium. NEC is a disease of prematurity and impaired gut perfusi on is thought to be responsible for the clinical picture. 4. The disorder shows a familial tendency. A young female.'short segment Hirschsprung's disease'. Clinical symptoms are usually present within the first few days of life with delayed passage of meconium. 3. Malrotation F. there is a highe r incidence in males (4:1) with an equal sex incidence in 'long segment Hirschsprung's' disease. Hirschprung's disease H. Aganglionosis may be restricted to the rectum or rectosigmoid region .

4. On auscultation there is a quintuple cardia c cadence. in the axillae. meconium plug -volvulus -malrotation -imperforate anus -failure of normal peristalsis . Coarctation of aorta E. Headache and epistaxis are symptoms. Attention is usually directed to the cardiovascular system when a heart murmur or hypertension in the upper extremities and absence. rectal passage of blood and peritonitis. On examination there is an absence of femoral pulses with a mid -systolic murmur heard over the back. discrete coarctation are asymptomatic.The site of obstruction is most commonly in 2nd part of duodenum with hypertrophy of the proximal duodenum. malrotation and biliary atresia.Hirschsprung's disease -intussusception may occasionally occur in the neonate Later in the neonatal period other conditions should be considered. leg fatigue and claudication. marked diminution. including pyloric stenosis. back. On auscultation there is a sharp holosystolic murmur heard along the left sternal border. This is a case of coarctation of the aorta. CXR shows a figure 3 sign in the left upper mediastinum secondary to hypoplasia of the aortic arch with poststenotic dilation of the aorta infra -coarctation. and spinous processes may become continuous if the lumen is narrowed sufficiently to result in a high-velocity jet across the lesion throughout the cardiac cycle. Duodenal atresia occurs in 1 in 10. 6-Options A. Ebstein anomaly Instructions: Match the correct physical sign/presenting symptoms with the lesion of congenital heart disease:1. Enlarged and pulsatile collateral vessels may be palpated in the intercostal spaces anteriorly.000 live births. Most children and young adults with isolated. Narrowing or constriction of the lumen of the aorta may occur anywhere along its length but is most common distal to the origin of the left subclavian artery near the insertion of the ligamentum arteriosum. A midsystolic murmur over the anterior part of the chest. large bowel atresia -mechanical obstruction -meconium ileus. 2. Failure of this normal process results in malrotation which results in a narrow mesenteric stalk which predisposes to midgut volvulus and intestinal obstruction. Babies present with vomiting. 5. and in cases of volvulus with abdominal distension. Babies present with bilious or non-bile stained vomiting. On examination there is a widely split fixed 2nd heart sound. Ventricular septal defect C. A 18 year old presents for a health checkup with no symptoms. On auscultation there is a continuous murmur. The extremities can feel cold and claudication with exercise may occur due to diminishe d blood flow from the aorta to the lower extremities. X-ray shows the typical 'double -bubble'. During embryological development the intestines herniate into the umbilical cord at 4 . Atrial septal defect B.PEDIATRICS EXAMINATION REVIEW Treatment usually involves an initial colostomy proximal to the aganglion ic bowel. Causes of bowel obstruction in the neon ate are: -atresia of any part of the gastrointestinal tract -duodenal. or posteriorly in the interscapular area. They then return to the abdomen and rotate in an a nticlockwise direction. jejunoileal atresia. A 17 year old presents with heada che. A 7 year old presents with breathlessness. This is followed by a definitive pull through procedure at 3 -12 months of age. Patent ductus arteriosus D. There is an association with Down's syndrome (30%) as well as cardiac anomalies.10 weeks gestational age. 4. A 3 year old presents with breathlessness and pedal oedema. Teaching Notes for Question 6 Theme: Congenital heart diseases Q1. An 18 year old presents with palpitations.Treatment is surgicalduodenoduodenostomy. 3. 5. 216 . or delayed pulsations in the femoral arteries are detected on physical examination. The upper extremities and thorax may be more developed than the low er extremities.

Q3. Hence a quintuple cadence is heard on auscultation.Ebstein anomaly (congenitally malpositioned tricuspid valve) is a cardiac defect in which the leaflets of the tricuspid valve are displaced into the right ventricle .5 x 109/L 2. pressure in the aorta is higher than the pressure in the pulmonary artery throughout systole and diastole thus a continuous murmur is heard.a fixed split S2. during inspiration. Drug-induced haemolysis Choose 1 217 . Disseminated intravascular coagulation D. In this condition. In an ASD. Prognosis depends on the size of shunt .3 mmol/L 17. Autoimmune thrombocytopaenia C. systolic ejection murmur at pulmonary area are some of the examination findings. Q5. Usually. who had accompanied him in the ambulance but subsequently left A and E before speaking to a doctor. told the paramedics that his friend had been well earlier in the evening and that he usually did not drink much alcohol or take any drugs. A friend. Infective endocarditis is more common. the atria are linked via the defect. ECG shows right axis deviation and features of right ventricular hypertrophy. therefore inspiration produces no net pressure change between them the timing of the pulmonic valve closure does not change with regards to aortic valve closure and has no effect on the splitting of S2 .4 x 109/L 0.3 g/L 3. Patent ductus arteriosus is usually asymptomatic but can present with left ventricular failure or pulmonary hypertension. Autoimmune haemolysis B. toxic granulation of neutrophils and platelet anisocytosis. The S1 is split.a portion of the right ventricle is atrialized.PEDIATRICS EXAMINATION REVIEW Q2. It is common in this condition for an atrial septal defect to be present and a fixed S2 is heard together with an S3 and an S4 . Atrial septal defect is usually asymptomatic until middle age. Features of left ventricular hypertrophy are found on examination and ECG.0 mg/L 140 mmol/L 6. In ventricular septal defect the left ventricular pressure is higher than that of the right ventricle during systole resulting in the characteristic holosystolic murmer. Q4.8 x 109/L 22. What is the diagnosis? Options : A. Investigations revealed: Haemoglobin White cell count Neutrophils Lymphocytes Monocytes Eosinophils Basophils Platelets Reticulocyte count Prothrombin time Fibrinogen D-Dimer screen Serum sodium Serum potassium Serum urea Serum creatinine Lactate dehydrogenase 8. 7-An 18-year-old man was admitted to casualty at 3 am having collapsed at a nightclub.8 x 109/L 0. there is an increase in venous return to the right ventricle which delays closure of the pulmonary valve. Large shunts cause high mortality from cardiac failure. S urgical ligation of the ductus gives good results. Right ventricular lift.1 x 109/L 32 x 109/L 12% 32 s 0.large defects cause disability by the age of 40.5 mmol/L 200 mmol/L 540 IU/L Activated partial thromboplastin time 90 s The blood film showed red cell fragmentation with polychromasia.6 x 109/L 25. widely split and fixed S2.0 x 109/L 0.

penicillin V. There is a firm mass with a poorly defined smooth lower border in the left upper quadrant of her abdomen. high neutrophil count and thrombocytopenia are all indicative of disseminated intravascular coagulation (DIC). Appendicitis E. Intussusception B. A 13 year old girl presents with a complaint of abdominal pain that she has experienced for the past two weeks. you note a redcurrant jelly stool. 3. 1. In addition the renal failure with high potassium levels (K) and lactate dehydrogenase (LDH) are suggestive of associated rhabdomyolysis. On examination.4 methylenedioxymethamphetamine (MDMA). 4. poor appetite and chronic watery. and complains of central abdominal pain. low fibrinogen. more than once or not at all. A 1 year old child has been noted to have bouts of screaming where he is doubled -up in agony from abdominal pain with a 60 minute pain -free period in between. Constipation H. His medications included cyclosporin. When you as k to see his nappy. anaemia. A per rectum examination is too tender to perform and an anal fissure is noted at 6 o'cloc k. the site of abdominal tenderness may shift with a change in patient position. a sore throat. is not toler ating fluids. which has worsened acutely today. The child with appendicitis is usually quiet with facial flushing. Six weeks later he presented with a rash. which she notes on the toilet paper. Teaching Notes for Question 8 Theme: Abdominal conditions in children 1. and on palpation the liver is non -tender. Nephroblastoma I. 2. History reveals a bright re d loss of blood per rectum with defaecation. Riedel's lobe of liver Instructions: Choose the single most likely diagnosis from the options listed above for each of the clinical scenarios described below. green diarrhoea. Thrombotic thrombocytopenic purpura Teaching Notes for Question 7 Theme: DIC and Ecstasy The coagulopathy. gener al malaise. as opposed to appendicitis. Her abdomen is soft on palpation and on auscultation there are normal bowel sounds. Strangulated inguinal hernia F. She is now asymptomatic. 218 . If the child is not admitted for observation. The longer history of this case suggests mesenteric adenitis as the single most likely diagnosis. A 4 year old boy is brought into hospital by his mother. On percussion of the liver there is no dullness. there should be careful discussion with the parents to observe for worsening symptom s despite analgesics and fluids. Meckel's diverticulum D. 8-Options A. co-trimoxazole and aciclovir. Today he has vomited twice. Hypertrophic pyloric stenosis G. Enlarged cervical lymph nodes on examination with a history of a previous URTI points towards mesenteric adenitis. Its clinical presentation mimics that of acute appendicitis. A 2 year old girl is referred urgently to clinic having presented to her general practitioner with haematuria on two occassions. with a trace of blood on his anus.PEDIATRICS EXAMINATION REVIEW E. It is most often associated with a viral infection. 9-A 18-years-old adolescent had a bone marrow transplant from his sister for treatment of acute myeloid leukemia. For the past 2 weeks he has had a cold. Pyrexia usually resolves rapidly with mesenteric adenitis. Mesenteric adenitis is caused by inflammation of the mesenteric lymph nodes. but an association with streptococcal infections of the upper respiratory tract has been reported. Unfortunately (for diagnostic purposes) appendicitis has few signs of peritonitis in the early stages. raised D-dimers. The causative agent is likely to be ecstasy/3. Mesenteric adenitis C. headache and nausea. and an abdominal mass. where the location of tenderness tends to be fixed. Each option may be used once. This will prevent a diagnosis of appendicitis being missed.

PEDIATRICS EXAMINATION REVIEW On examination. No organs were palpable. His pulse was 104 beats per minute (bpm) regular. The GP reported that she has lost about 2 stones over the past 2 months and that although she was preoccupied by preparing food.4 g/dL 90 fL 3. Cytomegalovirus infection D. Investigations revealed: Haemoglobin Mean corpuscular volume (MCV) White cell count Neutrophils Lymphocytes Monocytes Eosinophils Basophils Platelets Serum albumin Serum total bilirubin Serum alanine transferase Serum alkaline phosphatase Serum gamma glutamyl transferase What is the diagnosis? Options: A.8 x 109/L 0. she did not seem to be eating well. Heart sounds were unremarkable and his chest was clear.7 x 109/L 1.65 x 109/L 1. hepatitis (tender right upper quadrant (RUQ) and raised alanine transaminae (ALT) and bilirubin) and gastro -enteritis (diarrhoea – usually described as green and watery). Bowel sounds were active. Transfusion-associated graft versus host disease Teaching Notes for Question 9 Theme: Graft versus host disease He has all three of the main manifestations of acute graft -versus-host disease (GVHD) – dermatitis (macular rash affecting palms and soles). mainly affecting the palms of his hands and the soles of his feet. a diffuse macular rash was visible.0 x 109/L 0.1 x 109/L 147 x 109/L 36 g/L 64 µmol/L 90 IU/L 135 IU/L 40 IU/L 10-A 14 year old girl was referred to the local child psychiatrist by her GP who was concerned about her weight loss. Side-effect of immunosuppression E. The mother also informed the GP that despite losing weight her daughter was spending long 219 . Acute graft versus host disease B. His abdomen was soft apart from mild tenderness in the right upper quadrant. Neurological examination was normal. Her mother reported that she has been hording food in her bedroom. Clostridium difficile infection C. Choose 1 12. Blood pressure 112/74 mmHg.05 x 109/L 0.

patients may also have elevated growth hormone and cortisol levels. Clinical history alone. organomegaly. ECHO M. swab and blood cultures K. It is very rare in middle age and beyond. biopsy and enema H. Increased levels of growth hormone D. A 45-year-old man who was on a fast developed acute pain in his toe. 5. Bone scan C. 3. 4. In addition to amenorrhoea. Pelvic spring test G. Binging on fatty food C. joint X-ray J. The onset is usually in the teen years. self -induced vomiting and use of diuretics. Patients usually present with 15% or more body weight loss. 11-Options A. Absence of depressive features F. Which of the following supports a diagnosis of anorexia nervosa in this patient:Options: A. MRI D. more than once or not at all. Increased levels of cortisol E. Prognosis is variable. There is a small open wound. He also has a 4/6 murmur. A man has a bike accident and injures his leg. which is worse at night and relieved by dangling the hand over the edge of the be d and shaking it. The main psychopathology behind the illness is distortion of body image and morbid fear of fatness. A 45-year-old man presents with swollen. Thomas test F. Choose 5 220 . Rheumatoid factor. painful and stiff hands and feet and the symptoms are worse in the mornings. Use of diuretics Teaching Notes for Question 10 Theme: Anorexia Anorexia nervosa is a seriou s psychiatric disorder affecting girls and young women. Anti dsDNA and complement E. Patients routinely avoid fattening food and indulge in excessive exercises. A 5-year-old Asian refugee child develops high fevers. A 45-year-old woman presents with wrist pain. Neurophysiology Instructions: For each of the patients described below choose the single most likely option from the above list of options. Spinal X-ray and HLA profile B. Joint aspiration and microscopy I. Loss of over 15% of body weight B. Each option may be used once. Joint aspiration. The wound is treated but 2 days later he develops pain in his knee.PEDIATRICS EXAMINATION REVIEW time every day undertaking heavy exercises. 2. Sigmoidoscopy. Amenorrhea G. joint pains and a rash. It may also affect males who make less than 10% of all patients. 1. Treatment of the condition may include hospital admission to restore weight and psychotherapy. joint X-ray L.

This is rheumatic fever. swab and blood culture is needed here as this is a case of infection of the joint which may have gained entry from the wound. Diagnosis depends upon finding urate crystals in tissues and synovial fluid. In older age groups. Herpes simplex virus I. surgery. focal en cephalitis. lips. Q4: This is a case of infection caused by Epstein Barr virus which preferentially infects B lymp hocytes. Impetigo L. and desquamation of the skin of the fingertips. A 17-year-old African male presents with sore throat. erythema of the oral cavity. Although HHV -6A has not yet been associated with disease. conjunctivitis. painful and stiff hands and feet and the symptoms are worse in the mornings. Erythema toxicum Instructions: For each of the patients described below choose the single most likely option from the above list of options: Each option may be used once. palatal petechiae. HHV-6B has been associated with mononucleosis syndromes. A 6-month-old boy presents with a generalised rash after a bout of fever. 5. A non-immunised child of 5 years presents with catarrh. 3. 2. 12-Options A. Teaching Notes for Question 12 Theme: Fever with rash Q1: This is a case of Roseola infantum. It is characterised by unresponsiveness to antibiotics. Infection with HHV -6 frequently develops during infancy as maternal antibody wanes. He also has a rash which does not blanch on pressure. infection and diuretics. He also has the characteristic non-blanching rash of meningitis. lips. febrile. Scabies C. fever. 4. more than once or not at all. nonsuppurative cervical adenitis. It does not respond to antibiotics. a common illness characterised by fever with subsequent rash. lymphadenopathy. A mother rings A&E saying that her 3 -year-old boy has fever for 1 day and is drowsy. and 221 . Measles G. congested conjunctivae. fever. Varicella B. multi-system disease of children. He has some bluish white spots on his buccal mucosa. 4. He should have a full cardiac assessment. palatal petechiae. HHV-6B is also a major cause of febrile seizures without rash during infancy. starvation. HHV-6B can cause exanthe m subitum (roseola infantum). anorexia. It is an acute. 2. Rheumatoid factor and joint X -ray is needed here as this is a case of rheumatoid arthritis which presents with swollen. lymphadenopathy. 3. Joint aspiration and microscopy is needed here as this is a case of gout which is due to de position of sodium monourate crystals in the joints and may be precipitated by trauma. Joint aspiration. fever. and palms. Meningitis F. congested conjunctivae. malaise.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 11 Theme: Differential diagnosis of joint pains 1. Ebstein-Barr virus J. anorexia. erythema of the oral cavity. Roseola infantum K. and palms. Pityriasis rosea D. and splenomegaly. and changes in the skin and mucous membranes such as oedema. Neurophysiology is needed here as this is a case of carpal tunnel syndrome and neurophysiology to find the level of the lesion and assess axonal degeneration and the likelihood of improvement after surgery is essential. Q3: This is a case of meningitis as the child has a fever and has been drowsy. It presents with sore throat. malaise. Psoriasis E. A 3-year-old boy presents with oedema. Kawasaki’s disease H. 1. 5. The organism can and must be isolated. and (in immunocompromised hosts) pneumonitis and disseminated disease Q2: This is a case of Kawasaki disease (mucocutaneous lymph node syndrome.

Dystocia Instructions: Match the above options with the best descriptive statement from the choices given below:1.g. Koplik spots are pathognomonic grain of salt like spots on the buccal mucosa. Destruction of normally formed tissue 5.g. Dysplasia E.malformation. Diphtheria K. Infectious mononucleosis I. congenital heart defects b. 13-Options : A. conjunctivitis. e. Unusual forces acting on normal tissues . abnormal ear shape and size in many syndromes 14-Options : A. Influenza 222 . Disruption C. Herpes virus B. Chemical agent with a malign influence on development 2. fever. Deformation B. Meningitis M. Brucellosis E. Intrinsic defects in the pattern of development 4. Abnormal development of a structure caused by extrinsic forces 3.PEDIATRICS EXAMINATION REVIEW splenomegaly.g.deformation e. breech deformat ion sequence c. Hepatitis N. Teratogen F.g. e. Q5: This is a case of measles caused by RNA paramyxovirus spread by droplets. Abnormal organisation of cells in tissue . Tuberculosis F. Coryza L. Staphylococcus D. amniotic bands d. Candidiasis H. Rubella J. Poliomyelitis G.disruption. Malformation D. It presents with catarrh. Breakdown of normal tissue . Abnormal organisation of cells in tissue Teaching Notes for Question 13 Theme: Dysmorphogenesis Types of problems in morphogenesis include: a.dysplasia e. incubation period is 7 21 days. Streptococcus C. defective formation of tissue .

and fever. malaise. It presents with a mild macular rash and suboccipital lymphadenopathy. arthritis. A man who is on steroids for rheumat oid arthritis for a long period of time now presents with white patches in his mouth. 5. Q2: This is a case of Rubella caused by a RNA virus. Q4: This is a case of brucellosis which is common in the Middle East. Q3: This is a case of hepatitis. His mother gives a history of tonsillitis. sacro -ileitis and rash. A 35-year-old lady presents with myalgia. 2. nausea. He is found to have myocarditis. Small joint arthritis may also be present. hepatosplenomegaly. The following signs and symptoms are present: fever. arthritis. A diagnosis is made using blood cultures. Q5: This is a case of candidiasis which is commonly found in patien ts who have low immunity. Her bilirubin is raised. Diphtheriae usually presents with a brassy cough. toxaemia or conducting system inv olvement. Brucellosis is more commonly found amongst vets and farmers. A veterinarian presents with fever. anorexia.PEDIATRICS EXAMINATION REVIEW Instructions: For each of the patients described below choose the single most likely option from the above list of options: (Each option may be used once. The toxin may cause polyneuritis. The incubation period 14 -21 days. Teaching Notes for Question 14 Theme: Diagnosis of infections Q1: This is a case of diphtheria which is caused by corynebacterium diphtheriae. depression. depression. He was not immunised as a child. anorexia. malaise. Signature not verified! kamel hassan Digitally signed by: kamel hassan DN: CN = kamel hassan ORG = msd OU = 1 C = AD Date: 2007. vomiting. She also has abdominal pain. Shock may occur from myocarditis.30 19: 59:22 +02'00' Reason: I am the author of this document 223 . A 4-year-old boy presents to A&E with shock. vomiting. more than once or not at all. sacro -ileitis and rash. 4. 3.04. hepatosplenomegaly.) 1. A 15-year-old patient presents with a rash and suboccipital lymphadenopathy. tonsillitis and a false membrane over the fauces. This is evident from the fact that the patient has jaundice (yellow sclera) and pain in the region of his right upper abdomen.

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