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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 anticipate 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, differential
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

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

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

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

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

Occurs only after penetrating injuries to the heart. May be associated with formation of microthrombi.Refsum’s Disease: 1. May occur in liver disease 5. The prognosis is related to the degree of left ventricular outflow gradient 2. Is usually associated with a normal thrombin time 3. 3. Is associated with icthyosis 3. so should be treated with intravenous heparin 30-Cardiac tamponade: 1. 2. Is characterised by arterial hypertension.000. Prognosis is worse in children 3. 28-The following statements are true regarding Hypertrophic obstructive cardiomyopathy:1. 4. Progresses to dilated cardiomyopathy in 10% cases 5. Is an immediate response to a traumatic event 2. 5. It may be associated with aortic stenosis. 2. May lead to avoidance 4. Often involves ‘flashbacks’ 3. Is caused by the same enzyme as Hurler’s disease 4. mid-clavicular line.Post-traumatic stress disorder :1. Strenuous activity is proscribed in all patients 29. Is associated with feelings of detachment 26.PEDIATRICS EXAMINATION REVIEW 25. Is inherited as an autosomal dominant disorder 2. Should be treated with aspiration using a needle inserted via the second intercostal space. The central venous pressure is raised. 4. Is characterised by increase ECG voltage across all leads. It can present in the newborn.Disseminated intravascular coagulation: 1. 5.The following are true of adult polycystic kidney disease: 1. Liver cysts are seen in approximately 10%. The genetic mutation of type I has been linked to chromosome 16. Is affected adversely by radical weight change 27. The incidence is about 1:10. Is usually associated with a high fibrinogen level 2. 3. Runs of Non sustained ventricular tachycardia on Holter have a high predictive value for sudden cardiac death 4. Can sometimes be treated by play therapy 5. 9 . May be associated with cytomegalovirus or varicella zoster infections 4. Is one of the causes of retinitis pigmentosa 5.

Thalassaemia 3. Neonatal infection is more likely if the mother has concomitant HIV infection as well as HCV 4. Haemoplilia A 5. 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. Children are not infectious after the development of parotitis 4. It is caused by a DNA virus 10 . The proportion developing chronic infection and hepatocellular carcinoma are similar to HBV infection 3.4-glycosidic linkages 3. Deficiency of muscle phosphorylase. Venous thrombosis is a recognised complication 4. may result in an impaired exercise tolerance 5. Glycogenolysis in the liver releases glucose-6-phosphate into the blood stream 2. 1% of the world’s population is infected 2. The diagnostic test is Ham’s test 5. Adenosine deaminase deficiency 2. 5% 35-Phosphodiesterase inhibitors: 1. Cause peripheral dilatation 4. Cause relaxation of the diaphragm 5. Transforms into acute leukaemia in approx. Breast feeding should be discouraged in the HCV +ve mother 5. Branches in the glycogen molecule are produced by 1. The defect is a congenital abnormality of RBC production by the bone marrow 2. Infertility is a frequent complication of the orchitis associated with mumps 5. Cause bronchodilation 3. Diabetes mellitus 4. Sickle cell disease 34-In paroxysmal nocturnal haemoglobinuria:1. Adrenaline is an important stimulus for glycogenolysis in the liver during exercise 4. Hamoglobinuria is a characteristic finding 3. McArdle’s Disease results in fasting hypoglycaemia and hepatomegaly 32-Concerning Hepatitis C Virus: 1. The incubation period is 7-14 days 2. Decreases the pulmonary capillary wedge pressure 36-Regarding mumps infection:1. Have an anti-diuretic effect 2.PEDIATRICS EXAMINATION REVIEW 31-Regarding glycogen and its metabolism: 1. It is often asymptomatic 3.

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

4. Amitriptyline 4. cellulose and glycogen are broken down for absorption by salivary and pancreatic amylases. Metronidazole 5. Sweating can increase body evaporative water loss from 0. carboxypeptidases and amino peptidases are responsible for the breakdown of proteins for absorption. 5.5 litres per day and eliminate 2. The three known fat soluble vitamins A. Leukotriene inhibitors are associated with the development of Churg-Strauss . Chloramphenicol 45-Digestion and absorption: Which of the following statements are true? 1. Propranolol 5. chymotrypsin. About 9 litres of water enters the gut each day.PEDIATRICS EXAMINATION REVIEW 43-Chronic lymphocytic leukaemia :1. Digoxin 2. Is associated with splenomegaly at diagnosis in about 50% of patients 44-The following agents have good activity against Bacteroides fragilis: 1. Cefoxitin 4. Trypsin. Pethidine 3. Has a median survival of less than 5 years 5. The eosinophils to the lung are recruited from the bone marrow 2. Benzylpenicillin 2. 46-In asthma:1. Bronchial hyper-responsiveness (BHR) does not have to be present. 4. haemodialysis would be effective in removing drug in:1. Should be treated aggressively in the early stages of the disease 4. Is asymptomatic at diagnosis in less than 10% of patients 3. 5. Is associated with smear or smudge cells in the peripheral blood 2.4 Mcal heat from the body. 2. Steroids are contra-indicated if the patient is a diabetic 3. 3. 2. Blocking interleukin-5 maybe therapeutic 4. 5. Local humidity and air movement do not significantly influence heat loss. D and E are taken up by fat absorbing processes in the gut. 3. The glucose-containing polysaccharides starch. The skin is a good thermal insulator whose effectiveness is controlled through blood flow. 12 . The blood flow through skin is controlled through vasodilator sympathetic nerves whose firing rate is increased in response to heat. Changes in muscle activity are the main source of heat production for temperature control. 47-Regarding overdose. Salicylate 48-Heat inputs and outputs: Which of the following statements are true? 1.6 to over 4. Piperacillin / tazobactam 3. Amphipathic phospholipids and bile salts convert micelles into lipid droplets ready for absorption.

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

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

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

Leukotriene antagonists are a first line treatment in asthma 2. agitation and confusion having been to school. IL-1 causes pyrexia and induces protein synthesis in the liver 2. Inhaled glucocorticoids improve lung function in asthmatics 5. Imerslund syndrome 68-Which of the following is associated with common variable immunodeficiency? 1. Reporting unusual smells prior to the episodes 2. A normal EEG 70-The following statements are reagrding asthma: 1. Peak flow monitoring can be used in most children aged 5 years and above 71-Regarding cytokines:1. Skull radiograph shows 'pepperpot' skull 67-Hypochromic microcytic anaemia is seen in: 1. Which of the following features suggest complex partial rather than absence seizures as the cause of her problem :1. Provocation of the episodes by hyperventilation 3. Oxygen 16 . Alpha thalassemia 2. Interferon gamma causes increased expression of MHC class II molecules on antigen presenting cells 3. The first management step would be :1. Granulomatous lesions 5.PEDIATRICS EXAMINATION REVIEW 5. Susceptibility to recurrent parasitic infections 69-A 9 yr old girl is seen in clinic. Chemokines can induce the transmigration of T cells into inflamed tissues 4. Autoimmune disease 3. IL-2 s the main cytokine produced by naïve T cells 72-15-year-old teenager presented with few haehorrhagic spots. Autoimmune hemolytic anemia 4. Treatment can be divided into those that prevent and those that relieve symptoms 4. Normal levels of IgA 4. The diagnosis of asthma involves blood tests 3. Her teacher at school has reported that she has numerous episodes throughout the day of abruptly staring into space midconversation for a few seconds with rapid return of awareness. Inflammatory bowel disease 2. Folate deficiency 5. Sickle cell anemia 3. Can be used clinically to help treat infection 5. pulse 110 and BP 120/80. A good response to anti-epileptic drugs 5. Abnormal features on MRI scanning of the brain 4. Temp 39.

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

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

Upper airway obstruction 4. Decreases calcium and increases phosphate reabsorption in kidney 5. Marfan's syndrome 2. The overall effect is to increase calcium and phosphate level in the blood 90-Selective IgA deficiency:- 19 . 5. 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. 5. Increases calcium and decreases phosphate reabsorption in kidney 2. Has a false negative result of 15 . Fixed. Is a good means of detecting distal ureteric dilation. 86-Regarding P. 4. Has a false negative result of 10 . 3. 89-The following are true regarding parathyroid hormone actions: 1. Oral isotretinoin causes hyperlipidaemia 5.45% when looking for renal scarring. Females with acne commonly have elevated blood androgens 2. 4. Increases osteoclastic activity 3. Is a reliable means of looking for vesico ureteric reflux (VUR). Absent gag reflex with pharyngeal stimulation. Carinii infection warrants prophylaxis 4. Rickets 3.PEDIATRICS EXAMINATION REVIEW 1.20% when looking for renal scarring.50% when looking for VUR. Interleukin 1 alpha promotes comedogenesis 4. Rightward shift of the heart 85-Renal ultrasound: 1. 3. dilated pupils unresponsive to light. Has a false positive rate of 25 . It is recommended in all infants born to HIV infected mothers 2. 2.Carinii prophylaxis: 1. Oral steroids are a recognised cause 3. No motor response from painful stimuli. Exercise limitation 5. Intravenous Pentamidine is recommended 3. Increases 1. Previous P. Recommended when CD4 count drops below 1000microlitre 5. 2. No eye movement when 20 mls cold water is perfused into the external auditory meatus. Neuromuscular drugs should have been stopped for at least twelve hours. Oral candidiasis in HIV patients warrants Prophylaxis 87-The following are true about acne:1.25 dihyroxy vitamin D3 production in the kidney 4.

Will give a positive sickle test result at birth 3. Morphine. Is associated with strokes in 25% of patients by the age of 45 years 4. Is a direct continuation of the internal iliac 3. May present with autoimmune disease 4. 92-Regarding ketamine: 1. Can be co-inherited with alpha or beta-thalassaemia 94-The femoral artery: 1. Radiofemoral delay is the diagnostic sign 20 . 5. 5. especially in the neonatal period. Renal failure may arise. Alcohol. Is an indication for autologous haemopoietic stem cell transplant 5. Exercise. The superficial epigastric artery is a branch of this vessel 95-Coarctation of the aorta:1.PEDIATRICS EXAMINATION REVIEW 1. Is typical of Marfan’s Syndrome 2. IgG2 subclass deficiency may also be present with more severe infective complications 5. Has anti-sialogogue properties 4. May be used safely in patients with suspected raised intra-cranial pressure 5. Is more emetogenic than thiopentone 3. Is a potent respiratory depressant 93-Sickle cell disease :1. is very rare (incidence less than 1 in 10000) 3. lies in the adductor canal (Hunter’s canal) 5. In the upper third of the thigh the femoral artery is contained in the femoral triangle (Scarpa’s triangle) 4. Vomiting. 4. Is contra-indicated in reversible airways obstruction 2. Is associated with notching of the anterior ribs 4. Is caused by a mutation on chromosome 16 2. is associated with HLA-DR3 91-The following increase vasopressin secretion: 1. In the lower third of the thigh. Is typical of Noonan syndrome 3. 2. Runs as a single trunk from the inguinal ligament to the lower border of the popliteus 2. Is treated with iv sandoglobulin 2. Haemorrhage. 3.

Has a half-life in the plasma of approximately 24 hours 3. Seborrhoeic keratosis 100-Pulmonary surfactant 1. Synthesis is inhibited by thyroxine 5. the following suggest an underlying organic cause: 1. Actinic keratosis 5. G6PD deficiency 99-The following skin conditions are by definition associated with epithelial dysplasia: 1. presentation at age 10 2. Neurofibromatosis type I 2. Bowen’s disease of skin 3. pain localising close to the umbilicus 3. Synthesis is stimulated by glucocorticoids 101-Regarding sleep disturbance: 1. Nightmares are associated with appreciable body movements 3. Reduction in pulmonary flow can cause a decrease in surfactant production 4. Secretion can be suppressed by morphine 5. vomiting 98-The following are inherited in an autosomal dominant manner 1. 97-In recurrent abdominal pain in children. Sleep terrors last at least twenty minutes 4. Is a decapeptide 2. Sleep terrors occur in non-REM sleep 2. Secretion is increased by pain. family history of migraine 4. Is produced by type 1 alveolar cells 3. Galactosaemia 5. Xeroderma pigmentosum 2. pain during daytime only 5. Congenital adrenal hyperplasia 4. Nightmares are associated with intense vocalisation 21 . Basal cell carcinoma 4. Hereditary spherocytosis 3. Sleep terrors are associated with intense vocalisation 5. Is partly recycled by endocytosis into the synthesizing cell 2. Acts on the distal tubule of the kidney to increase reabsorption of water 4.PEDIATRICS EXAMINATION REVIEW 96-Anti-diuretic hormone (ADH): 1.

Bacterial endocarditis 3. 5. Chest pain 4. Is the same substance as EDHF. Vitamin B1 deficiency 3. Measured rather than calculated in a pH probe 5. Zinc deficiency 104-The following are true of COPD exacerbations:1. 3. Vitamin A deficiency 2. All patients should be given antibiotics 2. Lower than intracellular pH 3. pH less than 7. Most of the venous drainage of the heart occurs via the the Basian system of veins. 103-Factors associated with poor wound healing include: 1. 5. Is important in the maintenance of pulmonary vascular flow. 2. Operates by inhibiting PDE’s. A systolic 'click' on auscultation 5. Vitamin C deficiency 4. Cyanosis 2. 3. 4. The AV node is supplied by the left anterior descending artery in most of the cases. Is critical in increasing endothelin (ET-1) levels. There is one pulmonary vein from each lung that enters the posterior wall of the left atrium. 2. 106-Mitral valve prolapse is associated with:1. 4. Folate deficiency 5. There is a positive correlation in mortality and the number of smoking pack years 4.35 is an indication for antibiotics according to the BTS guidelines 5. Not essential to maintain ion distribution 4. Increases cAMP. Are associated both with rhinovirus and chlamydia pneumoniae infection 3. Equal to [H+] of 34-46 nmoles/L 2. Moraxhella catarrhalis is the most frequently implicated organism in exacerbation 105-The following statements are correct regarding the cardiac anatomy: 1. Right coronary artery is dominant in over three-fourth of cases.PEDIATRICS EXAMINATION REVIEW 102-Nitric Oxide (NO): 1. Foetal arrythmias 107-Normal arterial pH is: 1. The left atrium is larger in size as compared to the right atrium. Decreased in pregnancy 22 .

Recruitment of neutrophils 110-The following poisons are matched to the appropriate therapy:1. T3 is roughly 10 times more potent than T4 5. NK cell killing 4. Are detected by Northern blotting 113-Which of the following statements are true: 1. All steps in thyroid hormone synthesis are stimulated by thyroid peroxidase 112-Restriction fragment length polymorphisms (RFLPs):1. Nominal data may include blood group type 23 . CTL-mediated lysis 2.phenytoin 111-The following are true: 1. Are generated by digestion of DNA with bacterial enzymes 5. The following factors would be against a diagnosis of anorexia nervosa:1.phentolamine 4. Self-induced vomiting 5. Helper T-lymphocyte activation 3. The uptake of iodide into the thyroid follicular cell is by active transport 2. A history of laxative abuse 2. Are found every 100 bases 2. Oxidative burst within activated macrophages 5. The delusion that someone is trying to control her eating patterns 3.atropine and pralidoxime 3. T4 has a half-life of 24 hours 3. Are useful in linked-gene diseases 4.PEDIATRICS EXAMINATION REVIEW 108-A 16 year old girl is admitted with progressive weight loss for which no organic cause can be found. Discrete data may include serum glucose values 4. About 99% of circulating T4 is protein bound 4. Organophosphates . Are useful in diagnosis of multifactorial diseases 3. Beta-blocker . Discrete data may include number of children 3.hyperbaric oxygen 2. An excess of physical exercise 4. Data may be described as qualitative or quantitative 2.ethanol 5. Low plasma potassium 109-Defective Fas-FasL interaction leads to impairment of :1. Carbon monoxide . Tricyclic antidepressants . Ordinal data does not include tumour staging 5. Methanol .

Renin converts Angiotensin I to Angiotensin II in the kidney 2. Dermatitis herpetiformis 3. Molluscum contagiosum 117-The following are causes of lymphadenopathy: 1. The production of antibody is unaffected 4. Hand Foot and Mouth disease 4. Thyrotoxicosis 118-The following statements are true for the Renin Angiotensin System:1. Kawasaki disease 5. The development of gamma/ delta -T-lymphocytes is unaffected 3. 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. Lymphadenopathy is common because of the over-expansion of B-lymphocytes 115-The following statements are true about the mitral valve 1. It lies posterior to the sternum at the level of the fourth costal cartilage 3. Angiotensin II receptors are blocked by spironolactone 5. Rubella 2. Morning headache 2. Angiotensin II inhibits aldosterone secretion by the adrenal glands 4. Roseola Infantum 2. Macrophage function is impaired 2.PEDIATRICS EXAMINATION REVIEW 114-In an athymic child:1. Amyloidosis 3. Rheumatoid arthritis 5. Double vision 24 . Abdominal pain 3. Angiotensin II promotes renal arteriolar dilatation 3. The function of helper T-lymphocytes (Th) is affected while cytotoxic T-lymphocytes (Tc) activity is only moderately impaired 5. It is bicuspid 2. Losartan inhibits formation of Angiotensin II 119-Which of the following are common symptoms associated with raised intracranial pressure in children? 1. SLE 4. The associated papillary muscles are smaller than those of the tricuspid valve 4.

Legionnaires’ disease 2. Reduced lung compliance. Infection in pregnancy can result in fetal anemia 5. 25 . Rapidly repeating tonic-clonic seizure with not returning to full consiousness 124-Regarding Gross Motor Development.PEDIATRICS EXAMINATION REVIEW 4. It is the causative agent for exanthem subitum 2. Ataxia 5. Kick a ball at 2 years 4. Arthritis is a very common manifestation in children 3. Tonic-clonic seizure lasting 30 minutes with incontinence 5. Seizure 120-The following is true regarding endotoxin:1. Unresponsive patients who have been incontinent for 60 minutes 3. It is produced by some Gram-positive bacteria 4. Roll over by 2 months 2. It is secreted by bacteria during active infection 121-The following are zoonoses: 1. It is a protein 2. Hydatid disease 3. a normal child would be expected to:1. Ride a tricycle at 2 years 3. Chronic anemia can be a manifestation in some children 4. Cruise by 1 year 5. Hypercapnia. Salmonellosis 122-Regarding Parvovirus infections in children:1. All endotoxin has the same physiological effect 5. Not run until 2 years 125-The functional consequences of moderately severe mitral stenosis include: 1. It is heat-stable 3. Mouth twitching lasting 60 minutes with reduced GCS 4. Two or more fits within 30 minutes 2. Aplastic crises due to parvovirus infection in sickle cell disease usually requires bone marrow transplant 123-Definition of status epilepticus: 1. Lyme disease 4. Poliomyelitis 5. 2.

microangiopathic haemolytic anaemia is characteristic 5. Appears only after the 1st week of life 130-With regards to bronchiolitis. normal Na content is <20mmol/L 3. Causes an increase in chest wall compliance 3. RSV immune globulin is recommended in infants with cyanotic congenital heart disease to prevent severe RSV infection. 3. IL-6: induction of antibody secretion by plasma cells 5. Maintains the same surface tension for different sized alveoli 5. functional minimum urine output/day in 70kg male is 1400mls 128-In children with renal failure 1. Decreases the surface tension within an alveolus 2. In haemolytic uraemic syndrome. the following are true :1. normal pH is >5. Somatostatin reduces gastric motility 26 . CXR commonly shows hyperinflation.5 2. Anaemia of chronic renal failure is due to low plasma ferritin levels 3. IL-10: activation of B-cells 4. 5. IL-2 : activation induced cell death 3.PEDIATRICS EXAMINATION REVIEW 3. It is linked to maternal smoking. 126-Evaluate the truth of the following descriptions of cytokines and their functional activities:1. Is a glycoprotein 4. 4. Raised left atrial mean pressure. 131-The following concerning gastrointestinal hormones are true 1. 4. dialysis is indicated with rapidly rising sodium 2. Lymphopenia is common. protein content of 500mg/day is within normal range 5. IL-3 : growth and differentiation of bone-marrow stem cells 2. Protein intake should be restricted in chronic renal failure. Grade V vesico-ureteric reflux spontaneously resolves in only 10% 129-Lung surfactant 1. 4. 2. IL-12: induction of IFN-gamma secretion by activated T-cells 127-Urine 1. normal osmolality is 300-600 mosmol/kg 4. Ribavarin is curative. Reduced glomerular filtration rate. Reduced pulmonary vascular resistance. 5. In acute renal failure.

The vagus nerve is responsible for normal bronchial tone 2. Adenovirus 4. 133-The following organisms cause conjunctivitis:1.46 nmoles/L. Most of the alveoli in the adult lung are present at birth 4. Epstein Barr virus 2. 2. Decreased in pregnancy. Secretin inhibits pancreatic bicarbonate secretion 5.In beta thalassaemia: 1. The surface markings of the right middle lobe approximate to the axilla 5. Measured rather than calculated in a pH probe. Diagnosis can be made by Haemoglobin electrophoresis 4. The reticulocyte count is low 3. 137-Regarding lead poisoning:1. 2.PEDIATRICS EXAMINATION REVIEW 2. 5. Anaemic patients should receive regular iron supplementation 5. Meningioma is usually benign. 3. Splenectomy is contraindicated due to extramedullary haemopoiesis 136-The following statements concerning CNS tumours are true: 1. Not essential to maintain ion distribution. Glucagons increases GI motility 4. 4. Gastric carcinoma frequently metastasises to the brain. Gastric distension increased motility 3. Astrocytomas arise from the neuroglia. 4. Neisseria gonorrhoeae 134-The following statements are true: 1. 3. The major component of surfactant is dipalmitoyl lecithin 135. Glioblastoma multiforme is a benign neoplasm. Urinary lead concentration is the most useful indicator of exposure 27 . 5. Beta thalassaemia major presents at birth with profound anaemia 2. Haemophilus influenzae 5. Pancreatic polypeptide inhibits pancreatic secretion 132-Normal arterial pH is: 1. Cerebral metastases comprise around 70% of all brain tumours. Equal to [H+] of 34 . Chlamydia trachomatis 3. Lower than intracellular pH. There are 18 bronchopulmonary segments 3.

Absence of sweating in the limb 28 . Spinothalamic tract degeneration above the section level 5. 3. Anaemia 142-A patient develops low back pain which is progressive. Involves seizures that often occur at school 4. It is resected. Mobilisation of B-cells from the bone marrow 3. Can be successfully treated with carbamazepine 5. Is a common form of partial epilepsy 2. 5. Stellate ganglion. Phrenic nerve. temperature and preservation of light touch 4. Disseminated intravascular coagulation in systemic Gram-negative bacterial infection 4. 141-Which of the following would cause an increase in the carbon monoxide transfer factor? 1. Goodpasture’s syndrome 4. He is found to a spinal schwannoma. Extra-pulmonary restriction 2.PEDIATRICS EXAMINATION REVIEW 2. The superior intercostal artery. but division of the posterior spinal root occurs. Scalenus anterior. Haemodialyisis effectively eliminates lead 4. Production of acute phase proteins by hepatocytes 2. Loss of motor function 2. Loss of tendon reflex 3. Loss of pain. Has a poor prognosis 3. Is associated with basophilic stippling in white cells 138-Benign rolandic epilepsy :1. Induction of killing by cytotoxic T lymphocytes (CTL) 140-The neck of the 1st rib is related to: 1. Exercise 5. Resetting of the temperature threshold in the hypothalamus resulting in pyrexia 5. Churg-Strauss 3. Has a typical EEG with a 3 per second spike and wave pattern 139-Which of the following biological responses or consequences is mediated by the release of tumour necrosis factor (TNF)–alpha? 1. Which of the following is likely to result from this? 1. 2. In asymptomatic children with increased lead levels DMSA therapy is recommended 3. 4. The thoracic duct on the right. The half-life of the deep bone compartment is months 5.

Is sometimes related to poor parenting 3. Patients are susceptible to infection with bacteria and viruses 3. 4.PEDIATRICS EXAMINATION REVIEW 143-Autism :1. Is related to Sandifer Syndrome 5. Ganglion blockers 5. Progesterone 3. X-linked SCID is caused by a defect in the IL-2 receptor 4. 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. Blocking interleukin-5 maybe therapeutic 4. Can be treated successfully with psychotherapy 2. it is largely the kupffer cells in the liver that filter micro-organisms. Basophilic stippling of neutrophils is seen with asplenia. 145-Chronic lymphocytic leukaemia :1. The lymphoid function is carried out in the white pulp. 5. 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. 2. Is related to Fragile X syndrome 4. 3. Following splenectomy. Steroids are contra-indicated if the patient is a diabetic 3. 5. Is associated with smear or smudge cells in the peripheral blood 2. low dose penicillin & pneumovax are recommended. Is associated with splenomegaly at diagnosis in about 50% of patients 146-In asthma:1. Following splenectomy. Is asymptomatic at diagnosis in less than 10% of patients 3. Is related to Rett syndrome 144-With regards the spleen: 1. Suxamethonium 4. Cisapride 2. The eosinophils to the lung are recruited from the bone marrow 2. Bronchial hyper-responsiveness (BHR) does not have to be present. Patients have defective T-cell dependent antibody responses and defective cell-mediated immunity 2. Has a median survival of less than 5 years 5. Ondansetron 29 . The spleen is involved in the destruction of encapsulated organisms. Leukotriene inhibitors are associated with the development of Churg-Strauss . Should be treated aggressively in the early stages of the disease 4. 147-With regards severe combined immunodeficiency:1.

Should recognise his/her name 2. 13% of neonates with chromosomal abnormalities have associated CHD 4. Heterochromia 5. Approx. Loss of sweating over the ipsilateral face 4. Webbed neck and low hairline 4. Hypopigmented spots on the lips suggestive of Peutz-Jeghers syndrome 3. Can easily distinguish primary colours by name 3. Approx. Exophthalmos 154-An abnormal response to oral glucose loading is seen in:1. Partial gastrectomy 4. Hyoglossus 5. Levator veli palatini 4. Palatoglossus 3. Perianal skin tags 5. ASD’s account for 40% of all CHD 3.PEDIATRICS EXAMINATION REVIEW 149-A normally developing 18-month-old child: 1. Palatopharyngeus 153-Horner’s syndrome invariably produces :1. 2. Should be able to copy a vertical line 150-The following statements concerning congenital heart disease (CHD) are true:1. 151-The following are clues to the cause of an upper gastrointestinal (GI) bleed: 1. Ipsilateral ptosis 2. Malabsorption 2. Approx. Gestational diabetes 155-Metabolic influences on pH 2: Which of the following statements are true? 30 . Haematochezia due to peptic ulceration 152-The muscles of the soft palate are: 1. Plays in parallel with his/her peers 4. Acromegaly 3. 80% of children with Down’s Syndrome have CHD 5. Wilson's disease 5. VSD’s account for 70% of all CHD. Tensor veli palatini 2. 40% of patients with Turner’s Syndrome have coarctation and-or aortic stenosis. Should imitate observed actions 5. Purpuric rash suggestive of Henoch-Schonlein purpura (HSP) 2. Contralateral mydriasis 3.

A pituitary adenoma may cause a bitemporal hemianopia 156-The O2-Hb dissociation curve is displaced to the right in: 1. Chronic hypoxia due to cyanotic conditions 5. GFR can be measured using a thermodilution technique 159-In enuresis :1. A lesion of the optic chiasma will cause a homonymous hemianopia 2. First line treatment is imipramine 5. Methemoglobinemia 3. Ventilation is reflexly depressed by metabolic alkalosis. Metabolic alkalosis can be induced by vomiting from the stomach. Urine dipstick is a useful investigation 4. 3.HCT) 4. The pupillary light reflex is preserved in lesions of the optic nerve 4.1-0. A lesion of the left occipital lobe will result in a right homonymous hemianopia 3. In a 3 year old investigations must be carried out 2. Increased 2. Renal blood flow = plasma flow/(1 . 155. Kawasaki disease 5.2 3. Fetal Hb 2. Guillain-Barre syndrome 2. 4.3 DPG 157-Intravenous immunoglobulin (IVIG) is used in the treatment of:1. Increased lactic acid production 4. 2. Lesions of the optic nerve cause an enlargement of the blind spot 5. Increased central chemoreceptor firing rates decrease ventilation rates. Normal filtration fraction is 0. Metabolic alkalosis is the reduction in H+ concentration by any means other than decreased PCO2. The filtration fraction is the ratio of GFR to renal blood flow 2. Metachromatic leukodystrophy 158-In the kidney 1. 5% of 5 year old children are affected 3. Hyperventilation induced by metabolic alkalosis returns arterial pH to the normal pre-set points.PEDIATRICS EXAMINATION REVIEW 1.With regard to the visual pathways: 1. IgA deficiency 3. Conditioning is usually successful 31 . 5. Idiopathic thrombocytopenia purpura 4. GFR can be measured using Doppler ultrasound 5.

Hyperparathyroidism 3. May regress by incorporation of the tricuspid leaflet tissue 4. Absence seizures and generalised tonic-clonic seizures may occur 2. Hyperthyroidism 4. Cushing’s syndrome 2. Results in wide splitting of the second heart sound 3. Hearing abnormalities are common 5. Tricuspid atresia is a recognised association 5. Consciousness is altered 3. Relapsing polychondritis 5. EEG changes disappear in sleep 5. Hyperventilation often provokes a complex partial seizure 4. Septicemia 32 . There is an increased incidence of osteosarcoma 2. Pamidronate is a recognised treatment in children with this condition 4. Cardiac bypass is not necessary for surgical correction 164-Neostigmine: 1. Muscular dystrophy 2. Has been used in the treatment of SVT 2. Crosses the placenta 165-Recognized associations of systemic conditions and cataracts include: 1. Diagnosis is usually made by bone biopsy 163-Ventricular septal defects: 1.PEDIATRICS EXAMINATION REVIEW 160-Endocrine disorders that result in diarrhoea include:1. Galactosemia 3. Has an oral bioavailability of 50% 4. Is metabolized by plasma esterases 5. Carcinoid syndrome 161-Regarding complex partial epilepsy:1. Ethosuximide is an affective treatment 162-In osteogenesis imperfecta 1. Prolongs the duration of action of suxamethonium 3. Can present with heart failure in the neonatal period 2. Thyrotoxicosis 4. Blue sclera is diagnostic 3. Primary hyperaldosteronism 5.

The plantar grasp persists longer than the palmar grasp 3. Parenting techniques can be taught 5. Pyloric stenosis 171-Noradrenaline 1. Molecules consist of 21 carbon atoms. 170-The following diseases are inherited in an autosomal recessive manner:1. A newborn baby from 32 weeks gestation will turn towards a light 167-Glucocorticoids: 1. Iodine is taken up passively by the thyroid gland.PEDIATRICS EXAMINATION REVIEW 166-The following statements are true:1. Inhibit gluconeogenesis. T4 is released bound to thryroglobulin. 4. 5. Primary ciliary dyskinesia 3. 4. 3. Deep tendon reflexes are difficult to elicit at birth 4. Is a neurotransmitter at the pre-ganglionic sympathetic nervous system 2. Congenital adrenal hyperplasia 5. TRH is released by the anterior pituitary. Behavioural therapy is never useful 2. Is produced by the adrenal cortex 3. Thyroid peroxidase catalyses the formation of thyroxine. Cause retention of sodium. Are secreted from the zona fasciculata within the adrenal cortex. Methylphenidate (Ritalin) should not be given later than 4pm 4. 5. 2. May cause a reflex bradycardia when given by infusion 4. Insulin dependant diabetes 2. 2. Pharmacological intervention includes the use of stimulant drugs such as amphetamines 3. Reduce lymphocyte counts. It acts predominantly via the beta adrenoceptor 33 . 168-In the treatment of attention deficit hyperactivity disorder :1. A moro reflex with the fists remaining clenched is normal. Causes a rise in metabolic rate 5. Batten's disease 4. 2. 3. Poor appetite is a common side affect of methylphenidate (Ritalin) 169-Concerning the synthesis of thyroid hormones: 1. T4 is released in greater quantities than T3. An extensor Babinski response is normal in the first year 5.

2. The umbilical artery is a branch of the common iliac artery 173-The pancreas: 1. Hyperactivity in childhood 4. Is seen in Kartagener's syndrome 2. Is entirely retroperitoneal 2. 3. Is usually asymptomatic 3. Receives taste fibres from the anterior 2/3rd of the tongue via the greater petrosal nerve. May delay diagnosis of coeliac disease 4. Has bilateral cortical representation. Systemic lupus erythematosis 4. When lesioned below the level of the stylomastoid foramen will cause hyperacusis. Is supplied by branches of the coeliac artery alone 3. Is crossed by the attachment of the transverse mesocolon 4. 5. Has a sensory supply in the ear canal. Is related to the common bile duct posteriorly 174-IgA deficiency:1. Is crossed by the left renal vein 5. Hypotonia 176-The seventh cranial nerve: 1. 4. Maxillary hyperplasia 3. Nephrotic syndrome 2. 177-Strokes in childhood are associated with :1. Decrease in pulmonary artery resistance following closure of the ductus arteriosus 4. Receives taste fibres from the palate. Haemoglobin SS 5. Spontaneous recovery is a recognised feature 5. Intra uterine growth retardation 5. Affects both serum and secretory IgA levels 175-Abnormalities associated with foetal alcohol syndrome include:1. Activated protein C resistence 3.PEDIATRICS EXAMINATION REVIEW 172-In a healthy baby the transition from fetal to neonatal circulation involves:1. The ductus arteriosus closes in response to decreased oxygen concentrations 5. Moya moya disease 34 . Blood flow in the ductus arteriosus continues from right to left until its closure 3. Short palpebral fissures 2. Functional closure of the foramen ovale in the first 24 hours 2.

May be diagnosed on urine culture 3. Deficiency in glucose-6-phosphate dehydrogenase results in impaired motility of leukocytes 5. Antacids and prednisolone 2. Is a valveless system 2. Maintains a uni-directional flow of lymph towards the thoracic ducts 3. 4. Hepatitis C. K+ 2. Osmolarity 4. 5. The commonest inherited form of severe combined immune deficiency is x-linked 2. Malaria. May be complicated by thrombotic thrombocytopenic purpurea 5. Is treated with tetracycline 4. 2. HIV. The defect in Chediak-Higashi syndrome is in T-cell activation 4. Contains lymph draining from the liver and intestine with a protein concentration 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. Most recover within two weeks without treatment 2. 1.PEDIATRICS EXAMINATION REVIEW 178-The following statement(s) concerning inherited immunodeficiency syndromes are true: 1. Has a total lymph drainage of 6-8 litres of fluid/24 hours from interstitial fluid 5. 3. Hepatitis B. Cholestyramine and warfarin 3. Sugar 183-Co-administration of the following pairs of drugs results in reduced absorption of the latter:1. Sucralfate and phenytoin 4. Tuberculosis. Is more common around the New Forest 181-The lymphatic system: 1. 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. Amitriptyline and digoxin 35 . Protein 5. Na+ 3. The thoracic ducts drain into the venous circulation at the junction of the sub-clavian veins and internal jugular veins 4. 180-Leptospirosis:1. The incidence of lymphoid malignancy is increased in ataxia telangiectasia 3.

4. Causes muscle weakness. May be a sign of cerebral palsy 185-Conn's syndrome: 1. 2. Polyarteritis nodosa 36 . Systemic lupus 4. Two thirds of the cases are sporadic.Immune complex deposition in the kidney occurs in: 1. Require clonal expansion to mount an effective response 3. 186-Haemochromatosis:1. Is due to atrophy of zona glomerulosa. 3. Initiate firm attachment by binding to ICAM on the endothelium 188-With regards to aniridia:1. Goodpasture's disease 2. Is more common in females. Release cytokines wich affect the adaptive immune system 4. It is associated with partial deletion of long arm of chromosome 11. Vision is commonly decreased. Leads to sodium retention. Is associated with Jaccoud's arthropathy 3. Is always abnormal 2. Infective endocarditis 3. Leads to increased levels of plasma angiotensinogen 2. 5. Is more common in men 4. 2.PEDIATRICS EXAMINATION REVIEW 5. Are important for the resolution of inflammation 5. 5. Wilm's tumour is associated with familial aniridia. Is associated with HLA B8 5. Serum creatine kinase should be measured 4. Does not need to be investigated in a girl 5. 4. Is associated with vitamin C deficiency 2. 3. Have receptors that recognise bacterial cell wall constituents 2. 189. Is associated with a gene on chromosome 13 187-Phagocytic Cells :1. Metoclopramide and paracetamol 184-Toe-walking in an 18 month old child :1. It is usually bilateral. Spinal cord imaging may be indicated 3.

PEDIATRICS EXAMINATION REVIEW 5. The diagnostic test is subcutaneous endophonium. 2.Regarding myasthenia gravis in childhood: 1. 37 . Prevalence rate of thumb-sucking in children are universal across races. Vacterl association. 3. CHARG association. The neonatal form is always due to maternal antibodies. 4. Hypothyroidism. Thyroid dysfunction is a recognised association. 2. Left handedness is more common in exprem babies without cerebral palsy. Left handed children are more likely to have un intentional injuries. Sense of taste is present from birth. High dose regular short acting bronchodilators should be tried before inhaled steroids. 2. Patient education is emphasized. 4. Water vapor will cause inaccurate readings from a paramagnetic analyzer 191-The following syndromes/ associations are associated with congenital heart disease. Red light absorbs more oxyhemoglobin than deoxyhemoglobin 2. Alpha-1-antitrypsin deficiency. The disease can be confined to ocular muscles. Goldenhar 192. 4. 5. conjugated hyperbilirubinaemia may be caused by: 1. EMG shows decremental response to repetitive motor nerve stimulation. Septo-optic dysplasia. Leukotrene inhibitors are recommended in step 4. 3. 5. Plasmodium malariae infection 190-Regarding oxygen measurement: 1. All patients should be on inhaled steroids. 1. holt-Oram syndrome. 195-Persistant. 5. 193.The following statements regrd the treatment of asthma: 1. 25% of children are left handed. The clark electrode uses a silver/silver chloride cathode 5. 2. The isobestic point will vary with hemoglobin concentration 3. 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. 3. 194. 3. The readings from pulse oximeters are affected by anemia 4. 4. 2.

Toxoplasmosis. Night terrors occur in the stages of deep sleep. 2. 2. Increased 2-3 DPG. 199-The following maternal conditions are known to cause adverse effects on the neonate: 1. 4. High altitude. Hyperthyroidism. 38 . 3. 1. Cystic fibrosis. 2. Diabetes insipidus. 5. Haemolytic disease. 4. 5. 4. 3. 4. Convergent squint. Increased red cell volume and MCH. 5. 198-In young children. 2. 4. Raised temperature. 5. Breath holding attacks should be investigated with CT scan. 5. Breath holding attacks cause cyanosis. Wiskott-Aldrich syndrome. 3. Chronic mucocutaneous candidiasis. 3. 3. Alkalosis. 5. SLE. Chronic myeloid leukaemia. 197-The following involve deficiencies in T-cell immunity: 1. Di George syndrome. Bruton’s agammaglobulinaemia. Constriction of the pupil. 4. Diplopia. Sleep disorders are never treated with drugs. Ptosis. 196-Complete transaction of the oculomotor nerve results in : 1.PEDIATRICS EXAMINATION REVIEW 3. Loss of accommodation and light reflex. Cytomegalovirus infection. Chronic granulomatous disease. 2. Sleep walking occurs during REM sleep. 200-en uptake by haemoglobin is characterized by: 1.

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

The presence of HbsAg indicates acute infection only 5.Regarding hepatitis B virus (HBV): 1. HbsAg appears at approximately 6–10 wks indicating acute infection and then vanishes if virus has cleared. MARKERS AB: * Anti-HBc (IgM) . until 12 wks. * Anti-HBe appears next (10 wks). It is spread by blood Tx/IVDA/tatooists/sexually/vertically (most important worldwide).PEDIATRICS EXAMINATION REVIEW 1. They bind to B cell receptors 40 . Most sensitive test of continued viral replication is PCR for HBV DNA. if found. 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.The following statements are true regarding superantigens: 1. HBV is an RNA virus 2. Incubation of virus: 6 weeks – 6 months. HBV only replicates in the liver 3. * Anti-HBs appears last (12 wks).First to appear at approximately 8 wks. but persists in chronic infection (patient then becomes a carrier). indicates immunity. Indicates continued viral replication. * HbeAg appears at 6 wks and disappears at 10 wks as Anti-HBe rises ('seroconversion'). 2. They are very strongly antigenic bacterial polysaccharides 2. a full profile is requested. across Europe HBV and HCV cause approximately 50% hepatocellular carcinomas. indicates reduced infectivity. HBsAg is the first serological marker to appear 4. MARKERS AG: * the first screening Ix is HBsAg. Persistence of HbeAg indicates increased severity and infectivity. 10–15% prevalence in Africa/Far East.

excess stimulation of T cell proliferation occurs leading to abnormally high levels of IL-2. cerebral metabolism. 5. Is decreased at 72 hours in a normal person who is made to hyperventilate for 72 hours. Lead to excess stimulation of T cell proliferation and interleukin-2 production 4. azathioprine. chlorambucil. bleomycin. particularly toxic shock syndrome and staphylococcal food-borne disease. Shows a right shift in the autoregulation curve in patients with chronic hypertension. They are responsible for gram-negative toxic shock 5. Penicillamine 4. 3. Nitrofurantoin Teaching Notes for Question 3 Theme: Pulmonary fibrosis Causes of pulmonary fibrosis include amiodarone. They have a role in Gram-positive septic shock. NB: methysergide causes retroperitoneal. and cytotoxics (e. bleomycin. Shows a linear increase with PaO2. Teaching Notes for Question 4 Theme: Cerebral blood flow The CBF is affected by PaO2. Is increased in an acutely compensated metabolic acidosis. PaCO2. 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. with symptoms of nausea. fever and malaise. but not pulmonary fibrosis 4. 3. It is possible that they may also have a role in the pathogenesis and clinical features of Kawasaki disease.Cerebral blood flow: 1. and methotrexate).g. Chlorpromazine 3. ICP. melphalan. MAP.PEDIATRICS EXAMINATION REVIEW 3. pleural. Is unaffected by temperature. neurogenic factors and 41 .Causes of pulmonary fibrosis include:1. sulfasalzine. vomiting. cyclophosphamide. busulphan. Sulfasalazine 2. Because the binding to T cell receptors is indiscriminate. temp. Methysergide 5. 4. 2. pericardial and subendocardial fibrosis. nitrofurantoin.

in some cases. Every decrease in body temperature of 1 degree celcius will decrease the cerebral metabolism by 7%. cerebrovascular accidents. audible along the left sternal border. 11 percent at 20 years. and even death. overriding aorta) may be heard. 6. short murmur suggests that severe obstruction is present. As it is acute there will be no change in the CSF pH. 3. Is maintained using a depolarising agent such as suxamethonium. the magnitude of which is proportional to the severity of the desaturation. A right ventricular lift or tap is palpable. Patients with tetralogy of Fallot have cyanosis and digital clubbing. In childhood. Adults with tetralogy of Fallot have dyspnoea and limited tolerance of exercise. 5. 4. Allows improved intra-abdominal access. 5. Cerebral abscess. 2. An aortic ejection click (due to a dilated. including erythrocytosis. A right-sided aortic arch may be present. a soft. and lung markings are diminished. A systolic ejection murmur.PEDIATRICS EXAMINATION REVIEW drugs. 4. the severity of which is determined by the degree of obstruction of the right ventricular outflow tract. The peripheral pulses are normal. Is not necessary in ventilated patients. most patients die in childhood: the rate of survival is 66 percent at 1 year of age. 2. such patients may have sudden hypoxic spells. Studies have shown that although there is the initial cerebral vasoconstriction with the low PaCO2. 42 . A compensated metabolic acidosis is achieved via hyperventilation. The electrocardiogram shows right-axis deviation and right ventricular hypertrophy. cerebral abscesses or stroke. 6 percent at 30 years.5 Kpa but is fairly constant at levels above that. Teaching Notes for Question 5 Theme: Fallot’s tetralogy Most patients with tetralogy of Fallot have cyanosis from birth or beginning in the first year of life. and 3 percent at 40 years. seizures. as is compensatory erythrocytosis. Digital clubbing. Aortic ejection click. is caused by the obstruction of right ventricular outflow. since its pulmonary component is inaudible. The heart is classically boot-shaped. The first heart sound is normal. a systolic thrill (caused by turbulent flow across the right ventricular outflow tract) is palpable. Without surgical intervention. Block acetyl-choline receptors on muscle end plates. 1. hyperviscosity. the size of the heart is normal or small. 5. which will decrease the PaCO2. but the second heart sound is single. 4. characterised by tachypnoea and hyperpnoea. The intensity and duration of the murmur are inversely related to the severity of the obstruction of right ventricular outflow. and endocarditis. They may have complications of chronic cyanosis. In some patients. loss of consciousness. The CBF shows an exponential increase in the CBF at PaO2 levels of below 5. Arterial oxygen desaturation is evident. Such spells do not occur in adolescents or adults. abnormalities of haemostasis. this gradually normalizes within 72 hours despite the low PaCO2. with an upturned right ventricular apex and a concave main pulmonary arterial segment. 3. 40 percent at 3 years. 2. Single second heart sound. On radiography.Muscle relaxation during surgery: 1. followed by worsening cyanosis and. Right-sided aortic arch.The following are clinical associations of Fallot’s tetralogy: 1.

The commonest form of immunoglobulin deficiency is however. This presents between 6-12 months when the maternal antibody levels wane and the infant’s own humoral system is not yet fully developed. They cause hypotension via a drop in systemic vascular resistance 2. Selective IgA deficiency can also occur after phenytoin and barbiturate administration as well as post toxoplamosis. a chest radiograph is mandatory to exclude thymoma 5. 7-In hypogammaglobulinaemia. Side effects include histamine release. B-cell maturation is arrested at the pre-B-cell stage. Non depolarising agents(atracurium. 43 . * Although in the normal kidney. In XLA or Bruton’s agammaglobulinaemia. they reduce GFR and can precipitate acute renal failure and flash pulmonary edema. there is an increased incidence of lymphoma 3. In nephrotic syndrome. bradycardia and rarely hyperpyrexia. suggesting that Btk may be required to couple the intracellular signaling events mediated by the pre-B cell receptor. which of the followings are true? 1. thus they are contraindicated in patients with renal artery stenosis. which leads to hypotension (especially on the first dose) but may increase cardiac output in those with heart failure. Selective IgA deficiency is the commonest form of inherited Ig deficiency (Incidence about 1 in 800).vecuronium. the incidence is higher in patients with chronic lung disease suggesting that IgA deficiency may be associated with increased respiratory infections. * They are generally poorly protein bound and readily removed by hemodialysis.PEDIATRICS EXAMINATION REVIEW 5. Although usually asymptomatic. * They also prevent the breakdown of bradykinin. Selective IgA deficiency is the commonest inherited form 4. the loss of immunoglobulin in urine is the only cause of predisposition to infection 2. They are highly protein bound drugs 5. the susceptibility to pneumococcal infection is multifactorial and includes decreased immunoglobulin levels. which leads to dry cough in many patients. in a patient with renal artery stenosis. In patients over 40 years of age. In the normal kidney. they will increase renal blood flow and thus GFR. In common variable immunodeficiency. due to an increase in bradykinin levels 3. will cause an increase in renal blood flow 4. 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. measles or other viral infections.pancuronium) are longer lasting.ACE inhibitors: 1. May lead to a side effect of dry cough. 8. Side effects include histamine release. Teaching Notes for Question 6 Theme: muscle relaxants Depolarisng agents(suxamethonium) are short acting. transient hypogammaglobulinaemia of infancy. Some patients with “selective” IgA deficiency may also have deficiencies in IgG2 and IgG4. 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. immunosuppressive treatment and in those patients with immune complex mediated glomerulonephritis -a major factor is decreased levels of complement.

The GFR of a child reaches adult values (corrected for weight) by the age of one year 2. but functional maturation does not take place until later. In the proximal tubules.PEDIATRICS EXAMINATION REVIEW 9-Macrocephaly :1. 2. Graves disease. The glomerulus filters approximately 180 litres of fluid daily. there is near-complete absorption of glucose and amino acids 5. Formation of nephrons is completed at birth. The proximal tubule isotonically reabsorbs two thirds of the glomerular filtrate. Is associated with Cornelia de Lange syndrome 4. 10-Regarding normal renal physiology: 1. GFR is corrected for surface area. Hashimoto thyroiditis. but renal function is not necessary for normal intrauterine hemostasis. Is associated with high intelligence 3. Can be inherited 5. not weight. The fluid entering the DCT is iso-osmolar 3. 44 . 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 syndrome Neurofibromatosis Cornelia de Lange syndrome Osteopetrosis Angelman syndrome Tay Sachs disease Edwards and Patau syndrome Soto’s syndrome Rubenstein Taybi syndrome. the ultrafiltrate is modified: There is preferential absorption of sodium and bicarbonate and near complete absorption of glucose and amino acids in the proximal tubule. Under the influence of ADH. 11-The following conditions occur due to the presence of circulating antibodies: 1. 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. Glomerular filtration begins at 9 weeks of intrauterine life. there is increased permeability to water in the DCT 4. and during this process. Is defined as Head circumference > 1 SD above mean 2.

3. HIV cardiomyopathy is linked to CD4 count and HIV encephalopathy.No effect 14. 4.Increases emptying. 4 . there are circulating antibodies against acetylcholine receptors at the neuromuscular junction. Ranitidine 5. Myasthenia gravis. Mycobacterium tuberculosis 2. 12-The following can cause cardiomyopathy in HIV disease:1. this is due to pericardial effusions rather than cardiomyopathy. Regression of right ventricular hypertrophy occurs postnatally 5.5 . Hypoxia-induced vasoconstriction is the mechanism of closure of the ductus arteriosus 3. Motor neurone disease. Reidel's thyroiditis. HIV of the heart Teaching Notes for Question 12 Theme: Cardiomyopathy in HIV Cardiomyopathy can be caused by HIV itself. Cisapride Teaching Notes for Question 13 Theme: Gastric emptying 2. Inferior vena caval pressure falls after birth 45 . The aetiology of motor neurone disease is not clear. or other infections such as CMV. There is normally immediate closure of the ductus arteriosus 2. However. In myasthenia. CMV infection 5. 13.PEDIATRICS EXAMINATION REVIEW 3. Kaposi Sarcoma 4. Teaching Notes for Question 11 Theme: Auto antibodies Reidel's thyroiditis is probably viral in origin. The ligamentum teres is the remnant of the umbilical vein 4. Gastric distention 3. although TB. toxoplasma. Domperidone 4. 5. Supine posture 2. and lymphoma can cause an apparent cardiomegaly on a chest radiograph. Lymphoma 3.Gastric emptying is slowed by: 1. Kaposi sarcoma. A pericardial effusion carries a poor prognosis in HIV.The following is true regarding changes in the fetal cardiovascular system after birth: 1.

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. synthesis of bradykinin and prostacyclins is inhibited. As pressure in the systemic circulation rises.activation-induced cytidine deaminase. The subsequent macrophage dysfunction contributes . B-cell and macrophage function. In reduced sodium states. IgM levels are elevated but IgG and IgA levels are low. 46 . Therefore. the T-dependent B-cell response is defective. breathing results in a marked decrease in pulmonary vascular resistance.to the profound immunodeficiency seen in these patients Patients are susceptible to infection with extracellular bacteria as well as intracellular pathogens. thus causing closure of the ductus arteriosus.somatic hypermutation in B-cells In X-linked hyper-IgM.identifying the defective gene. although there are high levels of serum IgM which can be produced without T-cell help. CD40L/CD40 interaction is also necessary for optimal activation of macrophages by T-cells. Baroreceptors are the principal sensors for controlling sodium secretion and reabsorption. both increased activity of renal sympathetic nerves (leading to constricting afferent arterioles) and reduced glomerular capillary pressure. 5. Autosomal recessive hyper-IgM (type 2) is caused by defects in the CD40-activated RNA-editing enzyme. however neutropenia is common. . As the pO2 rises. 3. 2. The interaction of CD40L on T-cells and CD40 on B-cells is essential for B-cell activation and proliferation. Changes in aldosterone levels allow control of the re-uptake of 50% of sodium originally present in the glomerular filtrate.X-linked hyper IgM syndrome :1.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 14 Theme: Cardiovascular changes after birth Occlusion of the umbilical cord removes this low resistance capillary bed from the circulation. shunt through the ductus arteriosus reverses. Boys present with recurrent sinopulmonary infections and chronic diarrhea often caused by Giardia. isotype switching and formation of germinal centres (which are required for affinity maturation of immunoglobulin). 4. Renin secretion is controlled by the macula densa. rotavirus. This enzyme is required for immunoglobulin isotype switching and . IgA or IgE production. Is associated with normal levels of IgG 3.The ductus arteriosus can take up to 3 months to close in normal neonates 15. Causes defective B cell development 2.adenocarcinomas of the liver and biliary tract Laboratory studies usually reveal normal B. Can present with pneumocystis carinii infection 4. or Cryptosporidium. Treatment is supportive with IVIG and antibiotics 16. the loss of interaction between CD40 and its ligand results in impairment of T-cell. Diagnosis is confirmed by . inherited immune deficiency disorder resulting from defects in CD40 ligand/CD40 interaction. X-linked hyper-IgM (type 1) is caused by defects in the CD40 ligand expressed on activated T-cells. decrease Na+ and water excretion. liver cells contain angiotensinconverting enzyme. and there is a lack of IgG. Lungs secrete angiotensinogen.Control of absorption: Which of the following statements are true? 1. Campylobacter. These patients are particularly prone to opportunistic lung infections caused by Pneumocystis carinii. Is due to a defective CD40 ligand 5. kidneys secrete renin. There is an increased incidence of autoimmune diseases and malignancies including .and T-cell numbers. 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.

acidotic or hypoglycemic. Total body irradiation 5. 19.Regarding surfactant:1.Renal sympathetic nerves and intrarenal baroreceptor also control renin secretion · 17. cataracts may form as a result of many medical treatments. Production begins at 30 weeks 2. Surfactant production is suppressed if the baby is hypothermic.Angiotensinogen is from liver and the converting enzyme is in the lungs · . including : Systemic steroid administration Irradiation (including multiple CT scans. 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. Although dexamethasone is more commonly used. Testosterone stimulates surfactant production 4. Prolonged general anaesthesia 3. betamethasone has an identical effect on lung maturation. It is produced by Type II pneumocytes in the walls of the bronchi 3. theoretically) Chlorpromazine Thalidomide Busulphan Gold. 18. Massive blood transfusion 4. hence hyaline membrane disease is more common in males than females and more common in infants of diabetic mothers. Systemic steroids 2. Drash syndrome 47 . hypoxic. The hormones testosterone and insulin inhibit surfactant production.The following syndromes commonly have associated renal manifestations:1. Zellwegers syndrome 2. Betamathasone given to the mother improves surfactant production in the premature baby Teaching Notes for Question 18 Theme: Surfactant Surfactant production begins at 20-22 weeks. Production is increased during a stressful event like hypothermia 5. Unfortunately.A cataract may be induced accidentally by the following treatments: 1.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 16 Theme: Renal system . It is produced by Type II pneumocytes which are in the walls of the alveoli.

. Drash Syndrome .Bile: 1.Wide splitting of second heart sound may be evident in: 1. Is secreted by the bile canaliculi 2. 20. Live vaccines are contraindicted within 3 weeks of another live vaccine administration 2. Other contraindications to live vaccines include –steroid administration / immunosuppressive therapy/ severe egg allergy/ immunodeficient conditions. Meckel Gruber syndrome 4.and kidney. chronic diseases of heart. Contains salts that are only water soluble 5. bile pigments. This allows them to stabilise fatty emulsions. Often large ballotable kidneys with multiple cysts are present at birth. Normal children 48 .Aniridia. lecithin and cholesterol.Occipital encephalocoele and renal cysts. Wilm's tumour and genitourinary malformations. Severe febrile illness is a contraindication for DPT 5. antibiotic treatment are not contraindications to vaccines 22. Meckel-Gruber Syndrome .Nephropathy. * 80% of bile salts are reabsorbed.5 degrees C. Chromosome 11p deletion Teaching Notes for Question 19 Theme: Syndromes with renal involvement Zellweger's syndrome . * Bile salts are amphipathic. Live vaccines are not given to children on prolonged steroid therapy 4. and this is done in the terminal ileum. or . Contains NaHCO3 3. Wilm's tumour and ambiguous genitalia. persistent screaming. 21.PEDIATRICS EXAMINATION REVIEW 3. which means that they are both water and fat soluble. It is stored in the gall bladder and gall bladder contraction is stimulated by cholecystokinin (CCK). Most infants die in the first 6 months. lung .Contraindications to pertussis vaccination include hypersensitivity to the vaccine and a severe reaction such as shock. Salts are mostly reabsorbed in the large bowel Teaching Notes for Question 20 Theme: GI * Bile is produced by hepatocytes and secreted into biliary canaliculi. NaHCO3. 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. MMR can be given a week after administration of IV immunoglobulins 3. bile salts. Chromosome 11p deletion . Stable neurological conditions. Noonan's syndrome 5.Also called the cerebro-hepato-renal syndrome. fever over 40.serious neurological symptoms Malnutrition. * Bile consists of. Is released from the gall bladder in response to the hormone gastrin 4.The following are true regarding vaccine administration in children:1. minor illness. Lethal.

6 -Emergency treatment of hyperkalaemia . This can :be due to .3 .1 .pulmonary stenosis (delayed RV ejection) * VSD (increased RV volume overload).any delay in RV emptying as in RBBB (delayed RV depolarization) * .S2) Splitting of the first sound is not usually detectable clinically. Volume overload due to partial anomalous venous drainage (PAPVD) 3.4 .4 .3 .Widening of the QRS complex . potassium shifts back into the intracellular fluid Teaching Notes for Question 23 Theme: Hyperkalaemia -ECG changes of hyperkalaemia are . coarctation of the aorta) or * increased LV volume load (large PDA.Peaked T waves .Continuous salbutamol nebulisers or IV salbutamol .2 .5 . and * . however. IV calcium resonium should be commenced 4.though the machinery murmur tends to obscure * .Regarding hyperkalaemia :1.2 .IV sodium bicarbonate .1 .ventricular tachycardia .Prolonged PR interval . Continuous salbutamol nebulisers should be started immediately on diagnosis 3.5 49 . Tall P waves are seen on ECG 2.PEDIATRICS EXAMINATION REVIEW 2. when it occurs.Ventricular fibrillations .delayed LV depolarization (LBBB) * delayed LV emptying (severe AS.Glucose-insulin infusion .IV calcium gluconate . Severe aortic defect 4. Atrial septal defect 5.loads between the two atria occurs through the defect In the case of reverse splitting. it is .most often due to complete RBBB 23.ST depression . P2 occurs first and splitting occurs in expiration.Ion exchange resin . Calcium gluconate exchanges for potassium and enables its excretion 5.mitral incompetence (earlier aortic valve closure because of more rapid LV emptying) * Fixed splitting (no respiratory variation) is caused by an ASD where equalization of volume . 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 . If acidosis is corrected.

Symptoms include reliving of the trauma or event in ‘flashbacks’ or dreams. The two types of TCR are called TCR-1 (gamma-delta) and TCR-2 (alpha-beta).Calcium gluconate stabilizes the myocardium by antagonising the effect of potassium . Is an immediate response to a traumatic event 2. 95% die in the thymus 3. Medication also occasionally used. together with feelings of detachment or emotional numbness or blunting. May lead to avoidance 4. depending on symptoms encountered. or dramatic reenactment. 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. depression. Treatment: intensive individual therapy. puppetry. They are produced in bone marrow and paracortical regions of lymph nodes and spleen.T lymphocytes:1. and involves a complicated series of positive and negative selection steps coordinated by the thymocytes and dendritic cells within the thymus. 26. sometimes using play.recognise peptide fragments associated with MHC molecules 25. Family therapy sometimes useful. The TCR is a heterodimer of two chains (alpha and beta. TCR-2 cells .PEDIATRICS EXAMINATION REVIEW . Are derived from a precursor cell in the bone marrow 2.on the heart. storytelling. resulting in apoptosis of unwanted cells.Peritoneal or hemodialysis . 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 to a stressful situation or event. Can sometimes be treated by play therapy 5. 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 CD3. Maturation occurs in the thymus.Post-traumatic stress disorder :1. TCR-1 cells are thought to have a restricted repertoire and to be mainly non-MHC restricted.6 Note. It does not lower serum K+ levels 24. Is inherited as an autosomal dominant disorder 50 . Are usually activated by intact protein antigens 5. Lack of energy. anxiety.Refsum’s Disease: 1. All have the alpha-beta heterodimeric T cell receptor 4. panic attacks. Avoidance of people or situations associated with trauma. Often involves ‘flashbacks’ 3.

Cerebellar dysfunction also manifests more prominently with rapid changes in the patient's weight (particularly rapid weight loss) and hence it is recommended that a stable body weight is mantained. 5. . abetalipoproteinaemia. as well as renal impairment.000 of the population. 3. It is totally unrelated to Hurler’s syndrome. Teaching Notes for Question 27 Theme: Adult polycystic kidney disease APKD is an autosomal dominant condition. It can present at any time from early infancy. Ushers syndrome.and PKD2 has been located to chromosome 4 Clinical presentations include hypertension. The genetic mutation of type I has been linked to chromosome 16.diverticulitis and abdominal herniae 28-The following statements are true regarding Hypertrophic obstructive 51 . Over 50% have liver cysts. The disorder is associated with icthyosis (a desquamative skin disorder) and cardiac arrhythmias. plasma phytanic acid levels fall with an improvement in the neurological signs.PEDIATRICS EXAMINATION REVIEW 2. Other causes of retinitis pigmentosa are:Freidrich’s Ataxia.The following are true of adult polycystic kidney disease: 1.000. cerebellar ataxia. . It can present in the newborn. abdo mass or pain and polycythaemia. This occurs because the mutations are not absolute in eliminating function and because omega-oxidation can also reduce phytanic acid levels. By limiting intake of meat (animal fat) and green vegetables. Liver cysts are seen in approximately 10%. Is associated with icthyosis 3. haematuria. aortic incompetence. 27. Kearns–Sayre syndrome. . 2. cataracts. The incidence is about 1:10. Alports syndrome and Laurence-Moon-Biedl sydrome. It may be associated with aortic stenosis. The mutation is in the phytanoyl-CoA hydroxylase (PAHX) gene on chromosome 10p13. Toxic accumulation of phytanic acid causes adolescent onset progressive retinitis pigmentosa. UTI’s. Is caused by the same enzyme as Hurler’s disease 4. anosmia. The genetic mutation of type 1 is on the short arm of chromosome 16 (PKD1 gene). 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. and sensory and hypertrophic interstitial neuropathy with preservation of intellect. Is one of the causes of retinitis pigmentosa 5. 4. deafness.000 and 1:2. affecting between 1:1. Plasmapheresis has been used if the patient's clinical condition rapidly worsens.about 10% have pancreatic cysts and about 5% have Berry aneurysms Other associations include mitral valve prolapse.

its overall predictive value in identifying patients at high risk of sudden death is quite limited. Strenuous activity is proscribed in all patients Teaching Notes for Question 28 Theme: Hypertrophic obstructive cardiomyopathy In hypertrophic cardiomyopathy. Is usually associated with a high fibrinogen level 2. so should be treated with intravenous heparin Teaching Notes for Question 29 Theme: Disseminated intravascular coagulation * Disseminated intravascular coagulation (DIC) may be caused by bacterial infections. 3. In some. May be associated with cytomegalovirus or varicella zoster infections 4. * Liver disease can also cause DIC due to release of thromboplastins from damaged liver cells and reduced concentrations of antithrombin and protein C. although sustained VT is uncommon. 4. by severe falciparum malaria infections and by viral infections including CMV. * DIC may be associated with microthrombin causing skin lesions. Is characterised by arterial hypertension. There is no correlation betreen these two features however. Prognosis is worse in children 3.benefit as may implantation of a defibrillator 29. 2.PEDIATRICS EXAMINATION REVIEW cardiomyopathy:1. * Investigations show a prolonged thrombin time. HIV and varicella zoster. gangrene or cerebral ischemia but heparin therapy is usually not indicated as it can aggravate the bleeding tendency. The prognosis is related to the degree of left ventricular outflow gradient 2. a low fibrinogen and raised D-dimers or FDPs. thrombocytopenia. 52 . the degree of outflow obstruction and the risk of sudden death depend on the gene defect. Is usually associated with a normal thrombin time 3. The central venous pressure is raised. in particular gram-negative and meningococcal septicemia. May occur in liver disease 5. Occurs only after penetrating injuries to the heart. renal failure. Runs of non-sustained VT are found in one-fourth of patients of HCM patients. mid-clavicular line. 30-Cardiac tamponade: 1. it is a harbinger of sudden cardiac death. May be associated with formation of microthrombi. Runs of Non sustained ventricular tachycardia on Holter have a high predictive value for sudden cardiac death 4. Presentation in childhood signifies worse prognosis. however. Should be treated with aspiration using a needle inserted via the second intercostal space.Disseminated intravascular coagulation: 1. prothrombin time and activated partial thromboplastin time (aptt). Treatment with beta-blockers and amiodarone may be of . Progresses to dilated cardiomyopathy in 10% cases 5.

In muscle. Branches in the glycogen molecule are produced by 1. Conditions that stimulate glycogenolysis inhibit glycogen synthesis. Glucose-1-phosphate is then converted to glucose-6phosphate. Glycogen storage diseases: type I (von Gierke's disease) results from glucose-6phosphatase deficiency leading to failure of release of glucose from the liver. McArdle’s Disease results in fasting hypoglycaemia and hepatomegaly Teaching Notes for Question 31 Theme: Glysogen metabolism Glycogen. type II (McArdles disease) caused by a failure of muscle phosphorylase leading to normal blood glucose but impaired exercise tolerance. each residue being added from an activated donor molecule. Neonatal infection is more likely if the mother has concomitant HIV infection as well as HCV 4. raised JVP. Breast feeding should be discouraged in the HCV +ve mother 5. Massive hepatomegaly and fasting hypoglycaemia develop. Adrenaline is an important stimulus for glycogenolysis in the liver during exercise 4.PEDIATRICS EXAMINATION REVIEW 5. 1% of the world’s population is infected 2. starvation) and adrenaline (muscle.heart sounds and decreased voltages in all ECG leads 31-Regarding glycogen and its metabolism: 1. is found predominantly in liver and muscle as cytoplasmic granules. may result in an impaired exercise tolerance 5. Deficiency of muscle phosphorylase. Branches. Glycogenolysis in the liver releases glucose-6-phosphate into the blood stream 2. Is characterised by increase ECG voltage across all leads. are formed by 1. Coinfectin with HIV results in more rapid and severe progression to chronic liver 53 . Glycogen is a large branched polymer of glucose residues. Teaching Notes for Question 30 . the storage form of carbohydrate. In the liver. muffled .4-glycosidic linkages 3. and vice versa. Kussmaul’s sign. at every 10 residues or so. The rate of glycogenolysis is hormonally regulated by glycogenolysis (liver. The proportion developing chronic infection and hepatocellular carcinoma are similar to HBV infection 3.Theme: Cardiac tamponade Cardiac tamponade can occur after both penetrating and blunt injuries to the chest.4-glycosidic bonds by phosphorylase to produce glucose.6glycosidic bonds formed by a separate enzyme. Removal of glucose (glycogenolysis) occurs by cleavage of alpha-1. there is no phosphatase trapping glucose-6-phosphate within the cell.7% of the UK population is affected. It is spread by blood contact especially via blood transfusion and IV drug abuse. a glucose-6phosphatase allows passage of free glucose into the bloodstream.4-glycosidic bonds. UDP-glucose.6glycosidic bonds by a separate debranching enzyme. 32-Concerning Hepatitis C Virus: 1. exercise). Chains are formed by Alpha 1.1phosphate and by cleavage of a-1. and is characterised by hypotension. 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. Unlike hepatitis B infection is not concentrated in developing countries and 0. Sexual and vertical transmission occurs but is much less common.

33-Which of the following conditions would be suitable for somatic gene therapy:- 1. Thalassaemia 3. Adenosine deaminase deficiency. There is an increased risk of venous thromboses (esp. The disease may transform into acute leukaemia (in approx 5%). and chronic haemolysis may result in nephropathy. Hamoglobinuria is a characteristic finding 3.e. Diabetes mellitus 4. Decreases the pulmonary capillary wedge pressure 54 . in unusual sites such as saggital sinus or hepatic veins). Cause peripheral dilatation 4. Adenosine deaminase deficiency 2. and haemophilia fit these criteria. The diagnostic test is Ham’s test 5. as this form of gene therapy is unlikely to restore the levels of the protein to normal. Transforms into acute leukaemia in approx. 35-Phosphodiesterase inhibitors: 1. Crises of haemolysis can be triggered by infection. Haemoplilia A 5. Cause bronchodilation 3. The defect is a congenital abnormality of RBC production by the bone marrow 2. sometimes in combination with ribavirin but response depends on viral genotype. 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. Patients with diabetes mellitus require controlled release of insulin and gene therapy is unlikely to provide the answer. Sickle cell disease Teaching Notes for Question 33 Theme: Somatic gene therapy Somatic gene therapy (i. age. This results in intravascular haemolysis and haemoglobinuria. 34-In paroxysmal nocturnal haemoglobinuria:- 1. alcohol intake and degree of liver fibrosis and inflammation seen on biopsy. haemoglobinopathies in particular thalassaemia major.PEDIATRICS EXAMINATION REVIEW disease. Have an anti-diuretic effect 2. 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 complement. Islet cell transplantation into the portal vein is more promising as it preserves the microenvironment of the beta cells of the islet and allows controlled temporal release of insulin. Venous thrombosis is a recognised complication 4. Cause relaxation of the diaphragm 5. Hepatitis C can be treated with interferon alpha.The diagnostic test is Ham’s test (acidified serum lysis).

Family history of febrile convulsion following immunisation is a relative contraindication 55 . TNF-beta and are important for cell mediated immunity. TGF-beta 5. In general. 4. 5. Production of suppressive cytokines such as IL-10. The other important complications are CNS infection (aseptic meningitis) and pancreatitis. Both the LVEDP and PCWP decreases.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 35 Theme: Inotropes and vasopressors 1. Th2 cells secrete IL-4. The first dose of vaccines is given at a corrected age of 2 months in case of premature delivery 2. They are bronchodilators. They cause a weak diuretic effect. incubation period is 14-21 days. In 30-40% of cases the infection is asymptomatic. Helper T lymphocytes (HTL) are usually CD4+ T-cells. Children are not infectious after the development of parotitis 4. TNF-alpha . IL-5 and IL-10 and are important for humoral responses. More recently. IFN-g. 36-Regarding mumps infection:1. termed Th3 and Tr1 cells. increase the sensitivity to CO2 and increase diaphragmatic contractility. Promotion of immunoglobulin isotype class switching 3. further sub-populations of CD4+ T cells have been identified. 38-Regarding immunisation in the UK:1. Children are infectious for a few days before until a week after the parotitis. 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. Th1 cells secrete pro-inflammatory cytokines such as IL-2. It is caused by a DNA virus Teaching Notes for Question 36 Theme: Mumps Mumps is caused by an RNA virus. Inhibition of tubular Na reabsorption leads to a naturesis and may precipitate hypokalemia. The incubation period is 7-14 days 2. Production of perforin 4. Production of T cell growth factors such as IL-2 2. 2. 37-The following are known functions of T helper cells :1. It is often asymptomatic 3. Infertility is not common after orchitis. They also promote the production of opsonising antibodies. 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. They are ino-dilators. HTL can be sub-classified into TH1 and TH2 cells.3. Infertility is a frequent complication of the orchitis associated with mumps 5.

electrolyte imbalance. congenital heart disease. 40-The following statements are correct regarding Horner’s syndrome: 56 . rubella and polio are contraindicated in HIV +ve children 4. The next most common indications are metabolic liver disease and acute hepatic necrosis. Signs of chronic liver disease persist despite improvement in liver function 5. Live vaccines should be avoided within 6 months of termination of lymphoma treatment 5.PEDIATRICS EXAMINATION REVIEW 3. The most common indication is failed Kasai procedure 2. irrespective of prematurely. so does the child’s growth stigmas of chronic liver disease resolve. mumps. FK506 has superceded tacrolimus as an immunosuppressant 4. Premature babies should be immunised according to the recommended schedule from 2 months after birth. Patients on active chemo or radiotherapy or within 6 months of termination of such treatment are contraindicated for live vaccines. Prolonged inconsolable crying or high pitched screaming more than 4 hours is classified as a severe reaction to immunisation and is a contraindication to subsequent immunisation with same vaccine. renal dysfunction. 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. Compared with cyclosporin. chronic lung disease. such as thrombosis of graft vessels. However it should be noted that Polio is secreted for longer periods in HIV positive patients as compared to other people. Family history of febrile convulsion is not a contraindication to immunisation. Prolonged inconsolable crying to previous immunisation is a definite contraindication. asthma. 5-yr survival rates for children after orthotopic liver transplantation are around 30% 3. also may be an ominous early problem. 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. tacrolimus is associated with lower rates of acute and chronic rejection and reduced use of corticosteroids. As liver function improves. Measles. Teaching Notes for Question 38 Theme: Immunisations in childhood Prematurity.3. and Downs syndrome are associated with an increased risk of complications from infectious diseases and they should be immunised as a matter of priority. Early complications include fluid shifts. and hypertension. Organ rejection tends to occur after the early phase along with infection. Tacrolimus and FK506 are the same drug. Most recent figures are 81% at 1 year and 77% at 5 years. Steroids and either cyclosporine or tacrolimus are standard therapy to prevent rejection. Vascular complications. and 4 months afterbirth. Studies have shown that antibody response and adverse events are not significantly different in preterm and term infants immunised 2. 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). No harmful effects have been reported with MMR and polio in HIV +ve individuals. 39-Regarding paediatric liver transplantation:1.

It is associated with ipsilateral ptosis (paralysis of levator palpebrae superioris). May be associated with loss of sweating on the ipsilateral arm. * Alpha-adrenergic drugs. meiosis (paralysis of dilator pupillae muscle) and facial and upper limb anhidrosis. hypercarbia. Teaching Notes for Question 40 Theme: Horner’s syndrome. The IgE level is markedly raised in these patients 42-The following increases the pulmonary vascular resistance 1. histamine. 4. May be associated with ophthalmoplegia in the territory of the IIIrd cranial nerve. There is an association with coarse facies. There is a low incidence of atopy due to defective IgE function 4. kyphoscoliosis. Patients develop recurrent sinopulmonary and cutaneous infections that tend to be much less inflamed than appropriate for the degree of infection (cold abscesses). “Cold abscesses” occur 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. Anemia 4. The somatic component of the IIIrd nerve is unaffected and thus ophthalmoplegia is not a feature 41-In HIE or Job’s syndrome :1. It may be caused by injury to the superior cervical sympathetic ganglion. The PVR is decreased by 57 . Maximal expiration 3. The condition is X-linked 5. Prostacyclin Teaching Notes for Question 42 Theme: Pulmonary circulation The PVR is increased by * Hypoxia. Ptosis occurs because of loss of innervation to the smooth muscle portion of the levator palpebrae superioris. 5. Salbutamol 5. kyphoscoliosis. 2. There is a markedly raised IgE level 2.PEDIATRICS EXAMINATION REVIEW 1. 3. * 5HT. Pupillary dilatation occurs because of loss of innervation to the iris sphincter muscle. atopy and eczema. Horner's syndrome may be caused by any injury to the sympathetic chain in the neck. osteoporosis. vasoconstrictors. Maximal inspiration 2. acidosis. osteoporosis. and eczema 3.

bone marrow. Should be treated aggressively in the early stages of the disease 4. but it does not hydrolyse cefoxitin (cephalosporin with anaerobic activity). It is often a slowly progressive disease and should not be treated in the early stages. chymotrypsin. 2. cellulose and glycogen are broken down for absorption by salivary and pancreatic amylases. Smear or smudge cells are often seen in the peripheral blood which represent the fragile malignant cells which have been squashed as the blood film is spread. 5. and splenomegaly is seen in 50% at diagnosis. 45-Digestion and absorption: Which of the following statements are true? 1. carboxypeptidases and amino peptidases are responsible for the breakdown of proteins for absorption. Amphipathic phospholipids and bile salts convert micelles into lipid droplets ready for absorption. Median survival is 3-5 years. Benzylpenicillin 2. * Beta-adrenergic drugs. The lowest PVR is at the FRC 43-Chronic lymphocytic leukaemia :1. 4. Chloramphenicol Teaching Notes for Question 44 Theme: Anaerobic Infections B fragilis produces a â-lactamase which is sensitive to inhibitors. 44-The following agents have good activity against Bacteroides fragilis 1. D and E are taken up by fat absorbing processes in the gut. It also produces a cephalosporinase. Has a median survival of less than 5 years 5. About 9 litres of water enters the gut each day. The glucose-containing polysaccharides starch. liver and lymph nodes. 3.PEDIATRICS EXAMINATION REVIEW * Vasodilators. Metronidazole 5. Is associated with smear or smudge cells in the peripheral blood 2. but one third of patients die of causes other than their CLL. It is asymptomatic at diagnosis in over 30% of patients. Ach. Is associated with splenomegaly at diagnosis in about 50% of patients Teaching Notes for Question 43 Theme: Chronic lymphatic leukaemia Chronic lymphatic leukaemia (CLL) is due to an accumulation of mature lymphocytes in the peripheral blood. Cefoxitin 4. Is asymptomatic at diagnosis in less than 10% of patients 3. * Parasym nervous sys * Prostacyclin Effect of respiration: PVR varies with lung volume in a U-shaped curve. Piperacillin / tazobactam 3. spleen. The three known fat soluble vitamins A. Trypsin. Teaching Notes for Question 45 Theme: Gastrointestinal system 58 .

Bronchial hyper-responsiveness (BHR) does not have to be present. haemodialysis would be effective in removing drug in:1. betablockers for arrhythmias from tricyclics. and theophylline. Teaching Notes for Question 48 Theme: Thermoregulation Skin blood flow is largely controlled by sympathetic vasoconstrictor nerves. Leukotriene inhibitors are associated with Churg-Strauss syndrome and causality can not be excluded. Sweating can increase body evaporative water loss from 0. 4. Digoxin 2. 46-In asthma:1. tricyclics and lipid soluble beta-blockers have a very large VD.drugs The volume of distribution (VD) of the drug determines how effective dialysis will be in removing it from the body. carbamazepine. meprobamate. Vitamin K is also fat soluble and is also taken up by fat absorbing processes in the gut. Amitriptyline 4. Digoxin. Leukotriene inhibitors are associated with the development of Churg-Strauss . Blocking IL-5 will therefore be therapeutic.6 to over 4. quinine. which increase 59 . Haemoperfusion is effective for short and medium acting barbiturates. Micelles are smaller structures made from lipid droplets by these agents. naloxone for pethidine. Pethidine 3. The skin is a good thermal insulator whose effectiveness is controlled through blood flow. Salicylate Teaching Notes for Question 47 Theme: Haemodialysis. methanol. pacing/glucagon/isoprenaline for propranolol. Blocking interleukin-5 maybe therapeutic 4. More effective supportive measures are antidigoxin antibody/atropine/correction of hypokalaemia for digoxin. 5. 3. dapsone. 2.PEDIATRICS EXAMINATION REVIEW Amylases attack starch. Drugs for which haemodialysis is effective include salicylates.4 Mcal heat from the body. phenobarbitone. Activated charcoal should be given for aspirin. 5. Changes in muscle activity are the main source of heat production for temperature control. pathophysiology In asthma. The eosinophils to the lung are recruited from the bone marrow 2. 47-Regarding overdose. Local humidity and air movement do not significantly influence heat loss. Propranolol 5. The blood flow through skin is controlled through vasodilator sympathetic nerves whose firing rate is increased in response to heat. 48-Heat inputs and outputs: Which of the following statements are true? 1. Teaching Notes for Question 46 Theme: Asthma.5 litres per day and eliminate 2. opiates. ethylene glycol and lithium. cellulose is indigestible dietary fibre. by definition bronchial hyper-responsiveness must be present. phenobarbitone. Steroids are contra-indicated if the patient is a diabetic 3. and theophylline. IL-5 is produced by T helper cells (TH-2) and recruits eosinophils from the marow. chloral hydrate.

Condensation of chromatin varies at different stages of the cell cycle and with transcriptional activity. Regular nucleosomal spacing can be interrupted by transcription factors and other DNA binding proteins resulting in the creation of a nuclease hypersensitive site. All convective losses increase with air movement. H2B. 2. 4.e.two copies each of H2A. the half life of mRNA. Humidity controls evaporation and therefore the effectiveness of sweating. Some regions of DNA. Elaborate folding and protein–histone interactions organise the chromatin fibre (made up of the nucleosomal DNA) into a higher order structure with varying degrees of compaction. A fifth protein. They are known as trans-acting factors. Nucleosomes consist of eight proteins. but to this secondary release. together with the presence of alternatively spliced variants can affect the steady state levels of mRNA considerably. but can interact via associated factors. The higher order structure of chromatin plays a major role in gene regulation. histone H1. usually by stabilising the complex containing the RNA polymerase. Teaching Notes for Question 49 Theme: Gene regulation The association of double-stranded DNA with histones (and other non-histone) proteins forms chromatin. A major factor in dictating the level of messenger RNA (mRNA) in the cell is obviously transcription. They may not bind directly to the polymerase.g. because the protein is usually encoded on a different chromosome from the ones on which they operate. and are usually made up of DNA containing multiple repeat sequences. DNase I hypersensitivity sites are usually indicative of regions of active chromatin. Transcription factors control expression by binding to specific DNA sequences. making it accessible to digestion by DNase 1. for instance. The role played by length of mRNA is unknown but it is probably not a major factor determining stability of the transcribed sequence. Finally. Euchromatin refers to chromatin that is decondensed in interphase and is rich in actively transcribed genes. Secondly. 5. to the cell surface after an additional signal. Chromatin must be open for transcription to take place to enable the various transcription factors and the components of RNA polymerase to gain access. However. They alter chromatin structure by displacing the nucleosome histones and 'opening up' the DNA. the heterochromatic regions. The 60 . 49-In gene regulation:1. DNase I hypersensitivity is a method used to analyse the state of chromatin at a particular site on a chromosome. consist of very compacted nucleosomes. 3. H3 and H4 around which the DNA coils. helps pack the nucleosomes together to increase compaction. These areas are refractory to gene expression. proteins can be stored in the cytoplasm for release. DNAse hypersensitive sites are often associated with gene control regions such as promoters and enhancers as the activating transcription factors bound to these regions displace nucleosomes or alter their structure. and in some cases transcription factors may still bind DNA when the gene is not expressed.PEDIATRICS EXAMINATION REVIEW their firing rate in response to cold and decrease with heat. These nucleosome structures together with DNA form a repeat unit of approximately 200 nucleotide pairs and are very regularly arranged along the chromosome. while cis refers to the sequences that are immediately upstream of the gene i. are physically linked to the gene. Transcription factors are proteins that bind to specific DNA target sequences and then act to enhance transcription. once the mRNA has translocated to the cytoplasm and is translated. Thus DNase hypersensitive sites are often associated with active chromatin. DNA regions that are assembled into regularly spaced nucleosomes are more resistant to cleavage by this enzyme than are regions that lack nucleosomes due to the binding of proteins to the DNA. In this case. acetylation of histone tails opens the DNA structure and aids in transcription factor binding. The stability of mRNA is entirely dependent on its length. Specific sequences in the message seem to be involved in stabilisation. However it is important to remember that this is not always the case. efficiency of translation will be another factor determining the level of protein produced. The best example is that of the AU rich element (ARE). expression on the cell surface would not be related to transcription at all. Some proteins that bind to DNA act as silencers. Transcription levels determine protein levels. This is achieved by various protein complexes which use energy to displace histones or which modify histone tails. Chromatin plays no role.e. Trans means 'from elsewhere'.

Infection in early gestation carries a far greater risk of severe fetal disease. 51-Congenital CMV infection:1. In CMV intra-cranial calcifications are in a periventricular distribution.P with a velvety. 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. AREs have also been shown to play a role in the stabilization of mRNA . pigmented rash on her neck and axilla. Which one of the following investigations is the most relevant for her future management? 1.PEDIATRICS EXAMINATION REVIEW consensus sequence in the ARE is the AUUUA repeat element. 52-In systemic lupus erythematosis (SLE) 1. Hearing loss can deveolp gradually over the first 5 years 4. Ganciclovir is only used if there is CNS involvement. Fasting serum insulin/glucose ratio 3. Intracranial calcifications are seen in a periventricular distribution Teaching Notes for Question 51 Theme: Congenital CMV. Plasma leptin 5. Fasting serum cholesterol/triglyceride 2. She is the only child of her parents. 50-Sarah is eight years old and she was referred by her G.P was concerned about this and has addressed this issue with her and her parents. Congenital CMV occurs in approximately 1% of all live births and only 10% of these infections result in clinical symptoms. Only 10% of affected pregnancies have resulting long term sequele at birth 2. Diagnosis is by viral isolation from the urine 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. There is an increased liability to infections 3. chorioretinitis or pneumonitis. 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. Serum adrenocorticotrophic hormone (ACTH)/cortisol 4. Intravenous immunoglobulins are commonly used in severe disease 4. The complex mechanisms of ARE-mediated post-transcriptional regulation are still being revealed. The ARE triggers destabilization of mRNA when bound by certain proteins. Her weight is 49 kg and her G. The affected newborn should be treated with ganciclovir 5. Exacerbations may be associated with lymphadenopathy 2. Raynaud’s phenomenon is the commonest presenting symptom 61 . Severe clinical disease is associated with primary maternal infection in pregnancy. It is not itchy and she is not disturbed by it.

Prior to the introduction of USS examination. It is treated by routine nephrectomy 3. Unlike most connective tissue disorders.PEDIATRICS EXAMINATION REVIEW 5. Females are affected three times more commonly than men 3.000. Cyclophosphamide and high dose steroid is the first line in life-threatening flares.then nephrectomy is 62 . Calcinosis only occurs in CREST form of the disease 4. corticosteroids should probably be avoided in scleroderma as they may precipitate a renal crisis. 53-In scleroderma (systemic sclerosis):1. is incompatible with life. UnilateraI lesions have an incidence of 1:2500-1:4000 live births 4. Anti-Scl 70 antibodies occur in 50-60% of cases 5. The most commonly affected sites are the PIPs. Arthralgia and tiredness are almost universal. Little race effect. although progressive digital contractures may occur. It is a rare anomaly that presents as an abdominal mass in the neonate 2. MCDK is usually a unilateral disorder. Calcinosis (deposits of BCP) is seen in about 50% of patients with limited SS (CREST) and in 5% of those with diffuse SS. Rare in children. Penicillamine may delay the development of internal organ complications Teaching Notes for Question 53 Theme: Scleroderma Most patients' skin softens after 3-10 years of disease. There is usually 0% differential function on DMSA/ MAG3 imaging 5. Skin tightness may improve with time 2. As with several chronic inflammatory diseases. If hypertension does develop -which is rare . and nephrectomy was routinely performed. The female to male ratio is 3:1 (age 35-65). Bilateral disease. 54-Regarding multicystic dysplastic kidney disease: 1. The ANA is positive in about 95% of patients with either form. there is an increased risk of IHD. fingertips and knees (but can occur anywhere). 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. Anti-centromere antibodies are seen in up to 90% of patients with the latter. Evidence suggests that prophylactic nephrectomy in this condition is not indicated as complications are rare. Internal organ involvement is usually progressive. There is limited evidence that penicillamine delays the progression of cutaneous disease and interstitial lung disease. elbows. 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. However. Anti-topoisomerase (Scl-70) antibodies are only seen in 20-30% of patients with diffuse disease and in 10-15% of patients with CREST. it is now apparent that the condition is commoner than was previously supposed and unilateral disease often follows a clinically undetectable course. lymphadenopathy per se is commonly observed in flares. which has an incidence of 1:20. CDK was thought to be an uncommon disorder that presented as an abdominal mass during childhood.

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EXAMINATION REVIEW

warranted.
55-Regarding schistosomiasis:
1. Freshwater snails are the definitive host
2. Cercariae penetrate the skin and may lead to an itchy papular rash
3. Fever, urticaria and eosinophilia may be seen in the initial illness due to antigen-antibody
complex formation
4. Schistosoma haematobium infection may lead to rectal bleeding
5. Schistosoma haematobium infection may lead to an obstructive uropathy
Teaching Notes for Question 55
Theme: Schistosomiasis
Schistosomiasis is a trematode (fluke) infection.
Intermediate host - snails
Definitive host - humans
S. Haematobium
Urinary schistosomiasis
(Africa, Middle East)
Worms reside in vesical plexus
Eggs shed in urine
S. Mansoni
Intestinal/liver schistosomiasis
(Africa, SW Asia, Americas)
Worms reside in mesenteric veins
S. Japonicum
Eggs shed in stool
(China, Japan - more severe)
S. Intercalatum
(West/Central Africa)
S. Mekongi
(Mekong Delta)
LIFE CYCLE:
Eggs are shed in urine or stool into fresh water. Miracidia hatch and infect freshwater snails
(intermediate hosts). 4-8 weeks later cercariae emerge which penetrate skin of humans
and migrate via the lungs to the liver as a schistosomule. Adult worms develop in 1-3
months in the portal veins and migrate to their final habitats (see above) where eggs are
laid.
PATHOLOGY is caused by:
1. Invasion of cercariae:
* Swimmer's itch (papular dermatitis)
2. Katayama fever:
* Acute infection
* Fever, urticaria, eosinophilia, cough/wheeze, hepatosplenomegaly, diarrhoea
* Can treat with steroids
3. Eggs cause most of the pathology:
a) Eggs leaving gut/urinary tract cause bleeding
b) Eggs remaining cause granulomata and calcify:
* Urinary tract - obstructive uropathy, calcified bladder,
* squamous cell carcinoma bladder, pseudopapillomas
* GI tract - colonic polyposis leading to iron deficiency anaemia
c) Eggs in the portal tract cause periportal fibrosis (Symmers fibrosis) leading to portal
hypertension (but normal liver function)
d) Eggs can embolise to lungs (pulmonary hypertension, corpulmonale) or CNS (space
occupying lesion, cord compression)
4. Immune complex disease:
* Glomerulonephritis
* Nephrotic syndrome
DIAGNOSIS:
1. Direct microscopy of eggs in URINE (max at 12 noon, eggs have terminal spines remember terminal haematuria too) or STOOL (need to concentrate stools by formol-ether
method; eggs have lateral spines)
2. Immunodiagnostic tests - problematic: no indication of severity of infection; do not tell
past vs present infection; not species-specific; hi-tech.
TREATMENT:
Metriphonate - S. haematobium only; x3 doses

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Praziquantel - all species, single dose (sometimes repeated), no serious toxicityct - colonic
polyposis leading to iro
56-Regarding platelets:
1. They have a life span of approximately 10 days
2. Platelet production is controlled by specific cytokines
3. Platelets should be stored between 2 and 6 degrees C
4. Platelets can be stored for a maximum of 48 hours
5. 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, which are regulated by thrombopoietin, a
specific cytokine. They have a lifespan of 10-12 days thereafter being destroyed in the
spleen.
Platelet concentrates should be stored at around 20 degrees C and the pH kept between
6.2 and 7.8 - these conditions reduce the risk of a change in morphology of the platelets.
They should also be continuously agitated to encourage gas exchange.
One pool of platelets gives on average, an increment of 10 000 / ml.
57-Concerning Salmonella infection:
1. Results in a non-invasive enteric infection
2. Infection invariably occurs following oral ingestion of contaminated food products
3. Statutory notification is required
4. Antibiotic treatment is required for Salmonellosis
5. 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, elderly, sickle cell disease and those with reduced gastric acidity /
motility e.g. postgastrectomy, hypochlorhydria. Salmonellae are highly invasive but most
infections are mild and self limiting. Less than 5% of typhoid cases become chronic carriers
58-Carbon dioxide:
1. Does not affect plasma HCO3 concentration
2. Is transported in the blood as phosphate esters
3. Is transported in the blood in simple solution
4. Arterial CO2 rises during sleep
5. Arterial CO2 rises when living at an altitude of 9000 meters
Teaching Notes for Question 58
Theme: Carbon dioxide
1, 2, 3. CO2 is transported in blood in a variety of ways:
- Hydrolyzed to carbonic acid in the red cells, which then dissociates to hydrogen ions and
bicarbonate. Bicarbonate then diffuses into plasma in exchange for Cl ions.
- Dissolved in simple solution in plasma and RBC.

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- As carbamino-coumpounds in combination with Hb and plasma proteins.
4. Small rise of 0.4 kPa during sleep.
5. The low PiO2 at altitude causes hyperventilation, which causes the lowering of the
PaCO2.
59-Infantile hypertrophic pyloric stenosis:
1. Is more common in male babies than female babies
2. Is more common in the Afro-Caribbean population
3. Presents with projectile, bilious vomiting
4. Results in biochemical changes including low serum chloride, potassium and bicarbonate
5. Is surgically treated by excision of the pylorus muscle
Teaching Notes for Question 59
Theme: Infantile hypertrophic pyloric stenosis
Pyloric stenosis occurs more commonly in males with a ratio of 4:1. It is more common in
Europeans than in the Afro-Caribbean or Asian population. Vomiting normally commences
at 2-3 weeks of age, and is of feed, not bilious. It becomes more frequent and projectile in
nature. Typical biochemical findings are that of a hypochloraemic, hypokalaemic metabolic
alkalosis (raised serum bicarbonate levels) due to loss of gastric acid. On clinical
examination during a feed, an olive shaped mass can be felt in the right hypochondrium.
Ultrasound (US) examination confirms the diagnosis. Treatment is correction of the acidbase disturbance, followed by surgery. A Ramstedt's pyloromyotomy involves splitting of
the muscle layers.
60-The following are recognised causes of drug-induced immune thrombocytopenia:1. Chloramphenicol
2. Quinine
3. Penicillamine
4. Rifampicin
5. Heparin
Teaching Notes for Question60
Theme: Drug-induced immune thrombocytopenia.
All the above drugs can cause thrombocytopenia. However chloramphenicol, penicillamine
and phenylbutazone cause this by bone marrow suppression. 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. Patients present with
acute purpura and platelets often less than 10x10 9/l. Heparin also causes
thrombocytopenia through platelet aggregation.
61-The following statements are correct regarding urinary analysis in acute renal failure:
1. There is a good correlation between functional renal impairment and the findings on
urinalysis.
2. Pigmented 'muddy brown' granular casts suggest acute tubular necrosis.
3. Transparent hyaline casts are nonspecific and may be present in any case of ARF unrelated
to the specific aetiology.
4. RBC casts suggest primarily renal arteriolar diseases such as vasculitis.
5. Presence of proteinuria argues against acute tubular necrosis and suggests a primary
gomerular pathology.

65

PEDIATRICS

EXAMINATION REVIEW

Teaching Notes for Question 61
Theme: Acute renal failure
Pigmented 'muddy brown' granular casts are characteristic 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 epithelial cells of the loop of
Henle.
RBC casts ('active' urinary sediment) are characteristic of glomerulonephritis of any
aetiology.
Proteinuria of up to 1 gm per day is common in ATN; greater extent of proteinuria
suggests glomerular pathology.
62-Concerning meningococcal disease:
1. Is readily distinguished on clinical grounds from other infectious agents by the spreading
purpura
2. Presents in seasonal epidemics
3. Frequently co-presents with meningitis, making LP necessary at presentation
4. Mannose binding protein deficiency is a known risk factor for meningococcaemia
5. Ciprofloxacin is used for routine contact prophylaxis
Teaching Notes for Question62
Theme: Meningococcal disease
Other risk factors include splenectomy, terminal complement deficiencies (C6-C9).
Ciprofloxacin is used as an alternative prophylactic agent to rifampicin especially in
pregnancy or those taking the oral contraceptive pill.
63-Features of the systemic inflammatory response syndrome (SIRS) include:
1. Core temperature <35 deg.cent.
2. Respiratory rate >16/min.
3. Hypercapnia with PCO2 >6 kPa.
4. Tachycardia >90 bpm.
5. Metabolic alkalosis.
Teaching Notes for Question 63
Theme: Systemic inflammatory response syndrome.
Sepsis is defined as the development of a systemic inflammatory response syndrome. This
may cause progression to multi-organ dysfunction syndrome.
64-The stiff-man syndrome :1. Is a syndrome associated with chronic inflammation of peripheral nerves.
2. Is a predominantly autoimmune syndrome.
3. Is uniquely associated with GAD antibodies.
4. Is associated with fibrillation and myokimeia.
5. Is best treated by benzodiazepenes and baclofen.
Teaching Notes for Question 64
Theme: Stiff-man syndrome.

66

CT shows unilateral dilated ventricle. The cause in many cases is the presence of antibodies to glutamic acid decarboxylase (GAD). Infantile glaucoma (bupthalmos) commonly occurs. so severe cyanosis is rare early after birth. Aortic stenosis 2. Stiff-man syndrome can be treated with steroids and other immunosuppressants. It may involve one limb only (stiff leg syndrome). 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. The skull radiograph shows the 67 . Hypoplastic left heart syndrome 4. It occurs in 8% with unilateral lesion and 33% with bilateral lesion. over-riding of the aorta and a VSD. Are associated with cataracts 4. Typically have slit like ventricles on CT 5.PEDIATRICS EXAMINATION REVIEW The stiff-man syndrome (SMS) is an uncommon neurological disease. Refractory focal tonic-clonic fits lead to a progressive hemiparesis contralaterally. Benzodiazepines and baclofen are also helpful. characterised by symmetrical rigidity and spasm of predominantly axial and proximal limb muscles. 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. 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. anti–glutamic acid decarboxylase (GAD) antibodies. 65-The following conditions will present with cyanosis in the first week of life: 1. Fallot's pentalogy Teaching Notes for Question 65 Theme: Cyanosis in the first week. Similar antibodies may be found in temporal lobe epilepsy and in diabetes. 66-Sturge-Weber syndrome :1.Babies with tetralogy of Fallot usually have a patent ductus arteriosus at birth that provides additional pulmonary blood flow. ipsilaterally. but plasmapheresis or intravenous immunoglobulins are more effective. If GAD function is significantly decreased. Fallot's tetralogy 5.and is related to release of polysynaptic spinal and brainstem reflexes from inhibition. As the ductus arteriosus closes. Fallot's pentalogy includes an ASD along with the tetrad of infundibular pulmonary stenosis. Diagnosis is by MRI. Transposition of the great vessels 3. Develop hemiplegia on the ipsilateral side of the vascular lesion 3. hemiparesis and often low IQ. Left heart problems or outflow tract obstructions present as cardiac failure. RVH. The autoimmune aetiology is confirmed by the finding of insulin-dependent diabetes in 40% of patients. then the inhibitory neurotransmitter GABA is decreased and muscles become continuously stimulated by motor neurons. as it typically will in the first days of life. calcification and cerebral atrophy. Occurs in 50% of children with port wine stains in the ophthalmic division of the trigeminal nerve 2. the most common pathologic correlate. To further confuse matters. The spontaneous muscle activity in SMS is of central origin – not peripheral nerve dysfunction . including diabetes mellitus and seizures. cyanosis can develop or become more severe. have been associated with a wide range of human disease.

Autoimmune disease 3.think: -Decreased RBC folate -Hyper-segmented neutrophils -LDH All suggest folate deficiency. 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. Sickle cell anemia 3.RBC (x109/l) . this is no use in hemodilution states (i. 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 . Autoimmune hemolytic anemia 4.4 If DF is +ve = Fe deficiency If DF is -ve = beta-thalassemia trait Beware.PEDIATRICS EXAMINATION REVIEW intracranial calcification in the occipitoparietal lesions as ‘tram-line’ or ‘railroad track’ calcification. Imerslund syndrome Teaching Notes for Question 67 Theme: Hypochromic microcytic anemia. To differentiate between Fe deficiency and beta-thalassemia use: 'Discriminant function'(DF) = MCV(fl) .3. Alpha thalassemia 2. 68-Which of the following is associated with common variable immunodeficiency? 1.Primary X-linked recessive . Normal levels of IgA 4.(5 x Hb[g/dl]) . Inflammatory bowel disease 2. Hypochromic microcytic anemia is usually associated with: a) Fe deficiency b) Sideroblastic anemia: . Folate deficiency 5. Granulomatous lesions 68 . Imerslund syndrome is characterized by: a) Chronic mucocutaneous candidiasis b) Decreased PTH c) Proteinuria d) Decreased vitamin B12 absorption. 67-Hypochromic microcytic anaemia is seen in: 1.Malignancy . post hemorrhage or in pregnancy).Inflammation In sideroblastic anemia there is Fe. Treatment is with pyridoxine (vitamin B6). but accumulation in the RBC mitochondria results in stippling on the peripheral smear and ring sideroblast formation in the bone marrow.e.Secondary alcohol .

PEDIATRICS EXAMINATION REVIEW 5. Which of the following features suggest complex partial rather than absence seizures as the cause of her problem :1. longer duration. Treatments for asthma continue to evolve. Some T-cell functional assays may be abnormal. Inhaled glucocorticoids improve lung function in asthmatics 5. Susceptibility to recurrent parasitic infections Teaching Notes for Question 68 Theme: Common variable immunodeficiency. Features which make complex partial seizures. solid organs. IgM levels is also reduced in 50% of patients. Reporting unusual smells prior to the episodes 2. Autoimmune disease occurs in about 10% of cases. 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. Patients present with recurrent pyogenic infections. for example) and a poor response to antiepileptic therapies. In CVID. The diagnosis of asthma involves blood tests 3. viruses or Candida. haemolytic anaemia or SLE. usually immune thrombocytopenia. Granulomatous lesions have been reported in lymphoid tissues. the presence of aura and post-ictal phenomena. and skin and share many clinical properties typical of sarcoidosis. but it is the humoral immune deficits that usually present clinically. Leukotriene antagonists are a first line treatment in asthma 2. associated MRI abnormalities (mesial temporal sclerosis.(CVI) CVID refers to a spectrum of disorders in which cell-mediated and antibody deficiency are combined in variable amounts. Immunological findings are variable. rather than absence seizures likely. 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. Granulomas are a special feature of CVID and do not occur in other primary lymphocyte disorders. A good response to anti-epileptic drugs 5. and in some cases susceptibility to infections with Pneumocystis carinii. 69-A 9 yr old girl is seen in clinic. focal abnormalities or normal EEG (absences tend to have typical 3HZ spike pattern). There is an increased incidence of malignancy. include: Later onset. 70-The following statements are reagrding asthma: 1. Abnormal features on MRI scanning of the brain 4. patients have marked reduction in serum IgG and IgA. Provocation of the episodes by hyperventilation 3. A normal EEG Teaching Notes for Question 69 Theme: Childhood seizures Complex partial seizures may present with brief periods of reduced awareness. Peak flow monitoring can be used in most children aged 5 years and above Teaching Notes for Question 70 Theme: Asthma Asthma remains a clinical diagnosis without a highly specific or sensitive test. but the two main areas remain those of 69 . Blood tests are not usually helpful. 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.

CD8+ and NK cells. Total anomalous pulmonary venous drainage (TAPVD) 3.g. Tetralogy of Fallot 5. 73-The following congenital lesions are associated with cyanosis:1. Chemokines are chemoattractant cytokines and recruit immune and other haemopoietic cells to sites of inflammation Cytokines are increasingly used in the therapeutic arena e. IL1 mediates local inflammation. Temp 39.PEDIATRICS EXAMINATION REVIEW symptom relief and those that prevent attacks. At high concentrations. It is produced mainly by activated mononuclear phagocytes. but is also secreted by other cells e. 71-Regarding cytokines:1. agitation and confusion having been to school. epithelial cells and endothelial cells. pulse 110 and BP 120/80. Intravenous antibiotic 5. The first management step would be :1. At low concentration.g. Aortopulmonary window 2. IFN-gamma is produces by activated CD4+. IFN-gamma is used to improve macrophage function in patients with chronic granulomatous disease. and IFN-beta is used to treat multiple sclerosis. G-CSF is commonly used to treat neutropaenic sepsis. Intravenous fluid 3. IFN-alpha is used as an anti-viral agent. Oxygen 2. IL-2 s the main cytokine produced by naïve T cells Teaching Notes for Question 71 Theme: Cytokines IL1 is known as endogenous pyrogen. it causes pyrexia and enhances acute phase protein synthesis. Lumbar puncture Teaching Notes for Question 72 Theme: Systemic Sepsis The history suggets meningococcal sepsis. Interferon gamma causes increased expression of MHC class II molecules on antigen presenting cells 3. Chemokines can induce the transmigration of T cells into inflamed tissues 4. IV antibiotics must be given immedietly. Eisenmenger's syndrome 70 . but are not a first line treatment. Leukotriene inhibitors have a useful role in some patients. Glucocorticoids can improve lung function with regular use. Coarctation of the aorta 4. 72-15-year-old teenager presented with few haehorrhagic spots. It is a potent activator of macrophages and promotes the differentiation of Th1 cells. Can be used clinically to help treat infection 5. bronchodilators and glucocorticoids respectively. IL-1 causes pyrexia and induces protein synthesis in the liver 2. Throat swab 4.

Bradycardia 2. Amyloidosis.PEDIATRICS EXAMINATION REVIEW 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. 76-The following are consistent with myxedema: 1. Eczema). Malignancy. IgA and IgE levels are often low 5. Platelets are of normal size. As well as impaired cell mediated immunity (with lymphopenia) there are low levels of IgM.which plays a role in cytoskeletal organisation and possibly intracellular signalling. Cardinal features are thrombocytopenia. Metastatic cancer. There is an increased risk of malignancy 4. Microvilli on T-cells appear abnormal. normal levels of IgG and high levels of IgA and IgE.g. Patients also have recurrent respiratory infections and are susceptible to infections with organisms e. In addition. or when complex anatomic defects. Pneumocystis carinii and herpes simplex virus. unassociated with RV outflow obstruction. 74-Causes of hypoadrenalism include: 1. which follows meningococcal septicaemia. The defect is due to a deficiency in the Wiscott–Aldrich protein. Severe sepsis can cause haemorrhage in adrenals. Inheritance is autosomal recessive Teaching Notes for Question 75 Theme: Wiskott-Aldrich syndrome Wiskott-Aldrich syndrome is an X-linked combined immunodeficiency. 2. 4. The presence of pulmonary and aortic valves and an intact ventricular septum distinguishes this anomaly from truncus arteriosus. Dry skin 71 . The aortopulmonary window defect is a communication between the ascending aorta and the main pulmonary artery.Bone marrow transplant is curative. 3. cyanosis may be caused by persistence of fetal pathways. Intolerance to heat 3. Platelets are not only few in number but are also small and these children can present with life-threatening bleeding. Severe sepsis. also called as Waterhouse Friederichsen disease. cause mixing of the pulmonary and systemic venous return. 5. Tuberculosis. but are few in number 3. Cushing's disease. Cushing's disease requires adrenalectomy for hyperfunction. Antibody responses to polysaccharide antigens are deficient. Immunodeficiency. 75-In Wiskott-Aldrich syndrome:1. The immunodeficiency is restricted to T cells only 2. Teaching Notes for Question 74 Theme: Hypoadrenalism Addison's disease leads to hypoadrenalism. eczema and immunodeficiency. (mnemonic TIME: Thrombocytopenia. There is an increased risk of lymphoid malignancy.

* The classical features are lethargy. CA-19. compromise must be sought. Alpha-FP is increased in hepatocellular carcinoma and in testicular germline tumours (esp. intolerance to cold. 2. Myopathy 5. Factor V Leiden mutation and protein C deficiency are associated with venous thromboses. and they are usually more useful for monitoring response to treatment or disease recurrence than primary diagnosis. atrial fibrillation and polycythaemia. Beta-HCG and choriocarcinoma. bradycardia. 77-The following tumour markers are strongly associated with the tumours shown: 1. * Drug metabolism and excretion are reduced. 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). Polycythaemia Teaching Notes for Question 78 Theme: Arterial thromboses Arterial thromboses may be associated with Behcets syndrome. delayed deep tendon reflexes. undetectable if the tumour was not present. Beta-HCG is raised in hydatidiform mole and choriocarcinoma and testicular tumours (esp. always being detectable if the tumour was present and present at a concentration directly proportional to the volume of disease present. 3. CEA is generally associated with colorectal cancer (also may be increased in other GI malignancies and in smokers). Protein C deficiency 5. CA-125 is associated with ovarian and CA-19. weight gain. Teaching Notes for Question 77 Theme: Tumour markers The ideal tumour marker would be specific to a particular tumour. Alpha-FP and hepatocellular carcinoma. Behcets syndrome 2. As no tumour marker fulfils these ideals. teratoma). and also sensitive. seminoma).9 and ovarian carcinoma. Atrial fibrillation 3. Factor V Leiden mutation 4. CA-125 and pancreatic carcinoma. CEA and colorectal carcinoma. greasy skin and a proximal myopathy. 79-Salbutamol: 72 .9 with pancreatic cancer. hoarse voice. so these patients often exhibit increased sensitivity to certain drugs including opioids. 78-Arterial thromboses are associated with:1. 5. 4.PEDIATRICS EXAMINATION REVIEW 4.

It can present with abdominal pain and vomiting 3. 81-Regarding Tanner stages of puberty: 1. Asymmetrical breast development is common in girls during puberty 5.5 . Clinical features include recurrent episodes of oedema of skin.diarrhoea and vomiting and the upper airway. Boys' genital development is described by 5 stages 2.PEDIATRICS EXAMINATION REVIEW 1. Can cause an increase in PEFR 2. These episodes last hours to days.Salbutamol causes bronchodilation and increases the PEFR and FEV1. This glycoprotein regulates the classical complement pathway by dissociating C1r2s2 from C1q. 80-Regarding hereditary angioedema: 1.It causes a shift of potassium ions into cells. C4 levels tend to be low during an attack 5.It interferes with this mechanism (hypoxic pulmonary vasoconstriction) hence an adequate FiO2 is required during its admin. It is due to deficiency of complement component C1 2. Has no effect on hypoxic-pulmonary vasoconstriction 4. Treatment is with either danazol (boosts C1 esterase inhibitor levels) or tranexamic acid (inhibits plasmin. hence the rise in insulin. there is increased consumption of complement components C2 and C4 with the production of fragments that mediate oedema. Attacks can be triggered by exercise 4. 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. May cause a rise in insulin release 5. which consumes C1 esterase inhibitor). surgery or exercise can trigger attacks.2 . 3 . Causes hypokalemia after prolonged use Teaching Notes for Question 79 Theme: Beta agonists 1. Has no effect on the FEV1 3. gut causing abdominal pain. Breast development stage 3 refers to breast buds 3. Deficiency of C1 esterase inhibitor also results in increased formation of bradykinin which enhances oedema by acting on vascular endothelium. Pubic hair in the prepubertal state is described as Stage 0 4. It may also increase the plasma concentration of free fatty acids and glucose. 4. In its absence. 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 73 . Stress.

The median age of menarche was 12 years and 11 months. This however.8 years). is far from popular with the girls.causing laryngeal oedema. Purified C1 inhibitor can be given in acute severe episodes and should be given pre-operatively should one ever be required. As the patients are heterozygotes they will produce very small amounts of C1 inhibitor. This suggests that there has been no decrease in the average menarcheal age in the past 3 decades. 74 . which can be stimulated with regular danazol (an anabolic steroid). vomiting & diarrhoea but no point tenderness iii) airway . No data has been collected in a UK population since the 1950s.resulting in abdominal pain. Sometimes treated with the anabolic steroid danazol 5.75 years) to 1950s (12. There was no difference related to social class or ethnicity. 4: Asymmetrical breast development is a common phenomenon and just needs reassurance. 82-Hereditary angioedema is:1. A consequence of C2 kinin production 3. Treated with regular C1 inhibitor infusions Teaching Notes for Question 82 Theme: Hereditary angioedema Hereditary angioedema is an autosomal dominant condition. downy hair III darker. Plasmin mediates the release of C2 kinin and therefore is amenable to blockade with e-aminocaproic acid as an alternative first line therapy. as it induces acne. circumscribed and noninflammatory oedema is mediated by the production of vasoactive C2 kinin. Inherited in an autosomal recessive fashion 2. The much talked about drop was from late 19th century (14.only nipple projects * Menarche is either 0 or 1. 5: A recent BMJ paper looked at a sample of 100 girls to establish the average age of menarche. The inhibitor of C1 is grossly lacking and the resulting acute. curlier and spreading IV adult type hair but not on medial thighs yet V adult * Male: Genitals Stage I prepubertal II scrotum and testes grow and scrotal skin coarsens III penis lengthens and testes grow IV penis broadens and glans develops. A cause of spontaneous life-threatening laryngeal oedema 4. * Pubic Hair: Stage I prepubertal II sparse.PEDIATRICS EXAMINATION REVIEW menarche). weight gain and amenorrhoea. Testes/scrotum darken V adult * Female: Breasts Stage I prepubertal II breast bud III breast and areola enlarge IV breast and areola enlarge to form mound above main breast V adult . It causes swelling (spontaneously or following trauma) of the following:i) skin -especially the face ii) intestine . choking and is potentially fatal.

The dura is adherent to the bone 2. 85-Renal ultrasound: 1. 84-Pectus excavatum is associated with: 1. and depth of anesthesia may need to be altered in response. Upper airway obstruction 4. but it may be habitual related to parental fears. 2. Has a false positive rate of 25 . subdurals follow the form of the cerebral hemispheres. It is also poor at detecting scarring. In many children. Arachnoid folds project inwards to form the falx cerebri and falx cerebelli 4. 3. * Extradural hematomas form a lentiform shape. Pia is responsible for production of CSF Teaching Notes for Question 83 Theme: Meninges * The dura's rich arterial system supplies the bone and dura.45% when looking for renal scarring. Whereas renal USS is good for detecting distal ureteric dilation. Exercise limitation 5. 4. 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. as this is often not present at the time of scanning. * Dural stimulation is very stimulating. it may be a manifestation of Marfan’s.20% when looking for renal scarring. Is a good means of detecting distal ureteric dilation. Marfan's syndrome 2. * The dura project inwards to form the falx. Pia stimulation during surgery is very stimulating 5.50% when looking for VUR.50%). But. The broad ranges given relate to various studies. 5. Rickets 3. Subdural hematomas form a lentiform shape 3. Has a false negative result of 10 . rickets or upper/lower airway obstruction. Has a false negative result of 15 . A link has been suggested between pectus excavatum and exercise limitation. but also indicate the large amount of operator dependence found with all USS examinations 75 .PEDIATRICS EXAMINATION REVIEW 83-Regarding the meninges: 1. Teaching Notes for Question 85 Theme: Renal ultrasound scan. the heart is shifted leftward. it is poor at detecting reflux (the false negative rate is 25 . Is a reliable means of looking for vesico ureteric reflux (VUR). MVP and WPW syndrome appear to be other abnormalities associated with pectus excavatum.

persistant oral candidiasis and progressive weight loss. erythromycin. 7% of individuals have acne until age 40. 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. Treatment: Progress stepwise from (1) Topical treatments (2) Oral tetracyclines (3) Second-line antibiotics e. Androgens promote seborrhoea. It is recommended in all infants born to HIV infected mothers 2.Carinii prophylaxis: 1. 76 . 3. 88-The following criteria must exist before a diagnosis of brain death can be made: 1. Drugs used are cotrimoxazole and aerosolized pentamidine. 87-The following are true about acne:1. Apart from physiological acne. Although more common in teenagers. IL-1 alpha promotes comedogenesis. Activity of this enzyme is high in sebaceous glands of acne-prone individuals. Comedones represent hyperproliferation of the sebaceous follicles. Interleukin 1 alpha promotes comedogenesis 4. dry eyes. Type 1 5 alpha reductase is an enzyme converts testosterone to dihydrotestosterone which binds to receptors in sebaceous glands increasing sebum production. Carinii infection warrants prophylaxis 4. 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. Oral isotretinoin can cause hyperlipidaemia and deranged liver chemistry. Females with acne commonly have elevated blood androgens 2.their sebaceous glands respond excessively to normal levels of androgens (end-organ hypersensitivity). and drugs such as oral steroids and anabolic steroids. Other side effects: headaches.PEDIATRICS EXAMINATION REVIEW 86-Regarding P. Recommended when CD4 count drops below 1000microlitre 5. 2. Neuromuscular drugs should have been stopped for at least twelve hours. Previous P. mood alterations. Oral steroids are a recognised cause 3.acnes) and (4) Inflammation. It is a teratogen. Dapsone has also been tried. acne occurs in pathological conditions of congenital adrenal hyperplasia. minocycline. hair thinning. doxycycline. muscle aches. Intravenous Pentamidine is recommended 3.g. but most females have normal levels of androgens . Oral isotretinoin causes hyperlipidaemia 5. Fixed. Absent gag reflex with pharyngeal stimulation. dry lips and skin. trimethoprim (4) Dianette (cyproterone acetate and ethinyloestradiol) in women (5) Isotretinoin. dilated pupils unresponsive to light. CD4 count less than the cut off for the age or less than 200 in all ages.

Autoantibodies (organ-specific and non-specific) are often found even in the absence of clinical disease. No motor response from painful stimuli. is associated with HLA-DR3 Teaching Notes for Question 90 Theme: Selective IgA deficiency Selective IgA Deficiency is the commonest immune deficiency (affects 1 in 500-700 people). DR3 and deletions of the C4A gene. IgG2 subclass deficiency may also be present with more severe infective complications 5. 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.g. but can be inherited in an autosomal dominant or autosomal recessive pattern.secreting plasma cells. Is treated with iv sandoglobulin 2. Teaching Notes for Question 88 Theme: Brain death An absent gag reflex must be assessed with bronchial stimulation via a tracheal catheter. Patients are often asymptomatic. Decreases calcium and increases phosphate reabsorption in kidney 5. Autoimmune syndromes (e. There is an association with MHC alleles HLA B8. The defect in this syndrome is a block in differentiation of IgA-expressing B cells to antibody. No eye movement when 20 mls cold water is perfused into the external auditory meatus.PEDIATRICS EXAMINATION REVIEW 4. Serum IgA levels are usually < 50µg/ml but serum IgG and IgM levels usually normal. 89-The following are true regarding parathyroid hormone actions: 1. Vomiting. Anti-IgA antibodies occur in 30-40% and these may cause anaphylaxis if transfusions are administered.25 dihyroxy vitamin D3 production in the kidney 4. If present. Increases 1. Treatment is symptomatic – antibiotics for infections 91-The following increase vasopressin secretion: 1. 90-Selective IgA deficiency:1. Some patients may have additional IgG subclass deficiencies. May present with autoimmune disease 4. It is usually sporadic. clinical features are usually mild – recurrent sinusitis and respiratory infections (mild) or chronic diarrhoea. Increases calcium and decreases phosphate reabsorption in kidney 2. 2. 77 . Sjogrens disease. 5. is very rare (incidence less than 1 in 10000) 3. thyroiditis) are associated . Alcohol. pernicious anaemia. Neuromuscular drugs should have been stopped for at least 24 hours before assesment can be made. rheumatoid arthritis. Increases osteoclastic activity 3.

Is associated with strokes in 25% of patients by the age of 45 years 4. This is why the sickle test. Is more emetogenic than thiopentone 3. 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. 93-Sickle cell disease :1. is often not positive until 6-9 months of age and cannot be guaranteed until 78 . The following decrease vasopressin secretion: * Decreased plasma osmolality. It can be co-inherited with alpha or beta thalassaemia. Exercise. Morphine. * Angiotensin II. Alcohol also reduces the secretion of oxytocin. Haemorrhage. * It causes more post-op nausea and vomiting than thio and propfol (but less than etomidate) and cholinergic stimulation leads to increased saliva production. * Nausea and vomiting. * Pain. which detects the presence of sickle haemoglobin. 4. 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. Is contra-indicated in reversible airways obstruction 2. exercise and standing. * It causes increased cerebral blood flow.PEDIATRICS EXAMINATION REVIEW 3. either for induction or as a 3rd line treatment of an acute attack in a ventilated patient. * Alcohol. morphine and carbamazepine. stress. Is an indication for autologous haemopoietic stem cell transplant 5. May be used safely in patients with suspected raised intra-cranial pressure 5. * Increased ECF volume. Is caused by a mutation on chromosome 16 2. O2 consumption and ICP and frequently leads to emergence phenomena. Will give a positive sickle test result at birth 3. 5. Has anti-sialogogue properties 4. 92-Regarding ketamine: 1. * Decreased ECF volume. making it a good agent to be used in asthmatics. Is a potent respiratory depressant Teaching Notes for Question 92 Theme: Intravenous anesthetic agents * Ketamine is a mild respiratory stimulant and a potent bronchodilator. clofibrate. The alpha globin genes are coded on chromosome 16. Teaching Notes for Question 91 Theme: Vasopressin The following stimulate vasopressin secretion: * Increased plasma osmolality.

femoral artery The femoral artery is a direct continuation of the external iliac artery. Renal failure may arise. This group of left-sided obstructive lesion occurring together is termed the Shone complex.PEDIATRICS EXAMINATION REVIEW one year of age. M:F).Deep external pudendal . Mitral valve abnormalities (a supravalvular ring or prolapse) and subaortic stenosis are associated lesions. occuring in 25% of patients with homozygous sickle disease by the age of 45 years.Muscular . In the lower third of the thigh. The branches of the femoral artery are: . Has a half-life in the plasma of approximately 24 hours 79 . Is typical of Noonan syndrome 3. Is associated with notching of the anterior ribs 4. but 98% occur just distal to the origin of the left subclavian artery at the origin of the ductus arteriosus.Superficial external pudendal . Is a decapeptide 2. It is a feature of Turner’s syndrome and is associated with a bicuspid aortic valve in more than 70% of patients.Highest genicular 95-Coarctation of the aorta:1. Notching of the inferior border of the ribs from pressure erosion by enlarged collateral vessels is common by late childhood seen in posterior one third of the ribs. 94-The femoral artery: 1. The superficial epigastric artery is a branch of this vessel Teaching Notes for Question 94 Theme: Vascular anatomy. Patients with severe clinical manifestations of sickle disease have been treated with allogeneic bone marrow transplantation from an HLA-identical sibling. Is typical of Marfan’s Syndrome 2. The anomaly is commoner in males (2:1. Clinical problems include strokes and these are common. 96-Anti-diuretic hormone (ADH): 1. Is a direct continuation of the internal iliac 3. Radiofemoral delay is due to the blood flow to the descending aorta being dependent on collaterals. In the upper third of the thigh the femoral artery is contained in the femoral triangle (Scarpa’s triangle) 4.Superficial iliac circumflex . Autologous bone marrow transplantation is where the patients own bone marrow is replaced and would not be of benefit in this condition. especially in the neonatal period. lies in the adductor canal (Hunter’s canal) 5. Runs as a single trunk from the inguinal ligament to the lower border of the popliteus 2. 5. Radiofemoral delay is the diagnostic sign 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.Profunda femoris .Superficial epigastric .

* It acts on V1 receptors in the vascular smooth muscle giving it vasoconstrictor properties. Secretion can be suppressed by morphine 5. the more likely the presence of an organic cause. vomiting Teaching Notes for Question 97 Theme: Recurrent abdominal pain. There is however a high rate of spontaneous mutation and approximately 50% of affected individuals 80 . Certain features should. family history of migraine 4. It is also known by other terms including 'periodic syndrome'. alert you to this possibility.it is metabolised by the liver and kidneys. 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. the following suggest an underlying organic cause: 1. Congenital adrenal hyperplasia 4. and non-organic causes can only be suggested after the exclusion of the organic ones! 98-The following are inherited in an autosomal dominant manner 1. Neurofibromatosis type I 2. vomiting. exercise. presentation at age 10 2. dysuria. 97-In recurrent abdominal pain in children. Obviously. Galactosaemia 5. Acts on the distal tubule of the kidney to increase reabsorption of water 4. *Alcohol is a well-known inhibitor of ADH. Abdominal migraine is one of the variants of migraine headache. altered bowel habit. PR bleeding and constitutional symptoms are all worrying signs. Hereditary spherocytosis 3. pain during daytime only 5. This variant most typically occurs in children. however. stress. G6PD deficiency Teaching Notes for Question 98 Theme: Autosomal dominant inheritance Neurofibromatosis type I is inherited as an autosomal dominant disorder. They usually have a family history of migraine and go on to develop typical migraine later in their life. Recurrent abdominal pain is very common and seldom has an organic cause. These include presentation at less than 4 or over 15 years and pain that wakes the child at night. * Pain. sleep and morphine all increase ADH as does a rise in the osmolality of the extracellular fluid and hemorrhage.PEDIATRICS EXAMINATION REVIEW 3. pain localising close to the umbilicus 3. * Activation of V2 receptors in the distal tubule and collecting duct leads to increased water permeability via cAMP. having a half-life of 5 minutes in vivo .

which is not associated with dysplasia. the condition itself is not necessarily associated with dysplastic changes. Sleep terrors are associated with intense vocalisation 5. Hereditary spherocytosis is a dominantly inherited disorder with variable expression. * Basal cell carcinoma is an invasive condition. Actinic keratosis 5. Is produced by type 1 alveolar cells 3. Sleep terrors occur in non-REM sleep 2. Congenital adrenal hyperplasia and galactosaemia are inherited in an autosomal recessive manner. Synthesis is stimulated by glucocorticoids Teaching Notes for Question 100 Theme: Surfactant Dipalmityl-phosphotidyl choline is the main component of surfactant and is produced by Type-11 alveolar cells (granular pneumocytes).PEDIATRICS EXAMINATION REVIEW represent new mutations. 100-Pulmonary surfactant 1. at least in the early stages. 99-The following skin conditions are by definition associated with epithelial dysplasia: 1. 101-Regarding sleep disturbance: 1. and high levels of autonomic discharges. * Seborrhoeic keratosis is a common skin lesion. G6PD deficiency is X-linked recessive. Sleep terrors last at least twenty minutes 4. Although later on in the condition dysplastic lesions such as actinic keratosis may occur. 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 panic associated with intense vocalisation. * Bowen’s disease of the skin is by definition associated with carcinoma in situ of the epidermis. Bowen’s disease of skin 3. motility. Xeroderma pigmentosum 2. Seborrhoeic keratosis Teaching Notes for Question 99 Theme: Dysplasia in the skin. Synthesis is inhibited by thyroxine 5. Its half-life is 14 hours and its main function is to reduce the surface tension of the alveoli. 81 . * Xeroderma pigmentosum is a condition of defective DNA repair. Is partly recycled by endocytosis into the synthesizing cell 2. Reduction in pulmonary flow can cause a decrease in surfactant production 4. Basal cell carcinoma 4. * Actinic keratosis is a condition in which epidermal dysplasia is induced by sunlight. Nightmares are associated with appreciable body movements 3.

with subsequent production of high concentrations of NO. As a consequence of this. iNOS activity does not depend on intracellular calcium levels. Is important in the maintenance of pulmonary vascular flow. -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. security. child sits up or gets up with a panicky scream. the binding of calmodulin is essential for iNOS activity. There are three isoforms of nitric oxide synthase (NOS): neuronal NOS (nNOS). stimulation of these cells with cytokines or growth factors. or self esteem. of which the individual has very little recall.and iron and zinc clusters. Is critical in increasing endothelin (ET-1) levels. Is the same substance as EDHF. The dream experiences are extremely vivid and usually include themes involving threats to survival. NO modulates endothelin and maintains a low pulmonary vascular resistance.PEDIATRICS EXAMINATION REVIEW Nightmares are dream experiences loaded with anxiety or fear.it mediates the relaxation of vascular smooth muscle. 102-Nitric Oxide (NO): 1. DNA synthesis and mitochondria. Such a diverse range of potential targets for NO explains the large number of systems that utilize it as a regulatory molecule. hypertension and myocardial infarction. Importantly. In particular. NO has been shown to kill cells by disrupting enzymes involved in the Kreb's cycle. However. 2. Operates by inhibiting PDE’s. NO is also called endothelium-derived relaxing factor (EDRF). Increases cAMP. 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. 3. angiogenesis. NO is produced by a number of immune cells. 4. which mediates smooth muscle relaxation. The biological effects of NO are mediated through its reaction with a number of targets such as haem groups. All three isoforms can be found in a variety of tissues and cell types. producing NO in the process. but like the other NOS enzymes. can increase transcription of the iNOS gene. Often there is recurrence of the same or similar frightening nightmare themes. Its synthesis is catalysed by enzymes known as nitric oxide synthases (NOS). Insufficiency is believed to contribute to the pathogenesis of diseases such as atherosclerosis. cell signalling. nNOS and eNOS are constitutively expressed in mammalian cells and synthesise NO in response to increases in intracellular calcium and thus calmodulin levels. The NO formed in vascular endothelium diffuses to smooth muscle cells. sulfhydryl groups-to produce nitrosothiols. Oxygen and NADPH are co-factors in this reaction. immune defence and apoptosis etc. neurotransmission. These enzymes convert arginine into citrulline. and activates guanylyl cyclase producing cGMP. 5. 82 . Nitric oxide is a short-lived molecule (with a half-life of a few seconds). activated macrophages produce high concentrations of NO in response to LPS and interferon-gamma. endothelial NOS (eNOS) and inducible NOS (iNOS). Teaching Notes for Question 102 Theme: Nitric Oxide (NO): Nitric Oxide is a critical molecule involved in processes as diverse as vasodilation. often rushing to the door as if trying to escape. In most cell types iNOS protein levels are very low or undetectable.

There is one pulmonary vein from each lung that enters the posterior wall of the left atrium. 4. Are associated both with rhinovirus and chlamydia pneumoniae infection 3.pneumoniae and Moraxhella catarrhalis in causing bacterial exacerbations.35 is an indication for antibiotics according to the BTS guidelines 5. increased shortness of breath. NO is now known to be the physiological mediator of penile erections. Right coronary artery is dominant in over three-fourth of cases. All patients should be given antibiotics 2.g. Vitamin B1 deficiency 3.35) should receive antibiotics. followed by S. 104-The following are true of COPD exacerbations:1. slower wound healing. As part of its plethora of functions. increased sputum volume and increased sputum purulence. The left atrium is larger in size as compared to the right atrium. It enhances the effect of cyclooxygenases and stimulates the production of pro-inflammatory eiconosoids. wound). It is also involved in regulating apoptosis in neurons. Nitric oxide acts as a neurotransmitter in the central and peripheral nervous systems. Thus all patients with moderate or severe disease should be offered influenza vaccination seasonally. 105-The following statements are correct regarding the cardiac anatomy: 1. wound breakdown and dehiscence and death. There is a positive correlation in mortality and the number of smoking pack years 4. In addition with acute or chronic respiratory failure (pH less than 7. Vitamin C deficiency 4. it modulates firefly lighting! 103-Factors associated with poor wound healing include: 1. 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. there should be given antibiotics. low FEV1 chronic sputum production and the number of total pack years. pH less than 7. These may be manifest by increased infection rates (e. chest. 3.The following features are associated with increasing mortality with COPB exacerbations. 2. Trace element deficiencies often go hand in hand with malnourishment. The AV node is supplied by the left anterior descending artery in most of the cases. Folate deficiency 5. 83 . These are greater age. 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. Vitamin A deficiency 2. According to the BTS guidelines if a patient has two out of the following criteria.PEDIATRICS EXAMINATION REVIEW NO may act as a mediator of inflammatory processes. COPD exacerbations are frequently associated with influenza and rhinovirus infection. urinary.

There is an increased risk of infective endocarditis. Most of the venous drainage of the heart occurs via the the Basian system of veins. Lower than intracellular pH 3.to latecrescendo systolic murmur that continues to A2.they use this question a lot C.14sec after S1. The click is often followed by a mid. pH measured in pH probe E. 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’). Measured rather than calculated in a pH probe 5. Bacterial endocarditis 3. symptoms of diminished cardiac reserve predominate. 107-Normal arterial pH is: 1. 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. cerebellar infarcts. the incidence rises in patients with a systolic murmur. TIAs. A variety of non-specific symptoms may be present including palpitations. Chest pain 4.45 = [H+] 36-45nmol/L B. The sinoatrial node is almost always supplied by the RCA. pH 7. amaurosis fugax and retinal arteriolar occlusions all appear to occur more frequently. Venous drainage from the heart is mainly into epicardial cardiac veins. Decreased in pregnancy Teaching Notes for Question 107 Theme: Acid/base balance A. A systolic 'click' on auscultation 5. chest discomfort. Autonomic dysfunction occurs commonly in MVP and this has been suggested as the cause for the chest discomfort. and when MR is severe. Acute hemiplegia. Equal to [H+] of 34-46 nmoles/L 2. The unique physical finding in MVP is a systolic click at least 0.35-7. although the incidence appears to be extremely low in patients with a midsystolic click only.PEDIATRICS EXAMINATION REVIEW 5. Teaching Notes for Question 105 Theme: Cardiac anatomy The right atrium is larger in size than the left atrium in normal people. draining into the coronary sinus and then into the right atrium. Mitral valve prolapse is a common syndrome and the vast majority of patients are asymptomatic. postural hypotension. The 4 pulmonary veins drain into the left atrium. 106-Mitral valve prolapse is associated with:1. Intracellular pH = extracellular pH . a large majority remaining asymptomatic for many years. The outlook for MVP in children is excellent. In pregnancy. Foetal arrythmias Teaching Notes for Question 106 Theme: Mitral valve prolapse. Not essential to maintain ion distribution 4. Normal pH absolutely essential for enzyme function/ion distribution/protein stability D. Cyanosis 2. The following factors would be against a diagnosis of anorexia nervosa:- 84 . The ‘dominant’ coronary artery (CA) is the one that supplies the posterior part of the interventricular septum.

Oxidative burst within activated macrophages 5. Tricyclic antidepressants . and are responsible for the destruction of antibodycoated target cells. try atropine. superoxide anion and nitric oxide within activated macrophages and neutrophils upon phagocytosis.phentolamine 4. CTL kill target cells by Fas ligand-mediated apoptosis as well as by perforin-mediated cytolysis. Organophosphates . A low plasma potassium may result from laxative use or self-induced vomitting.hyperbaric oxygen 2. Examination findings include emaciation.PEDIATRICS EXAMINATION REVIEW 1. 109-Defective Fas-FasL interaction leads to impairment of :1. The delusion that someone is trying to control her eating patterns 3. Natural killer cells destroy cells by the release of cytoplasmic granules containing perforin and granzymes. glucagon infusion. brittle hair and nails and dry skin. A delusion of outside control implies psychosis. Carbon monoxide . A history of laxative abuse 2. 110-The following poisons are matched to the appropriate therapy:1. and temporary pacing. Defects in the NAPDH oxidase pathway leads to susceptibility to bacterial and fungal infections. especially in infancy and results in chronic granulomatous disease. Low plasma potassium Teaching Notes for Question 108 Theme: Anorexia Anorexia Nervosa most commonly occurs in young women. Self-induced vomiting 5.atropine and pralidoxime 3. It is characterised by an obsessive refusal to eat and altered body image.FasL Fas-FasL interaction leads to apoptosis of the Fas expressing cells. This process is catalysed by an enzyme complex NADPH oxidase and is important in killing the engulfed microorganisms. It has a mortality of up to 5%. this should be excluded when making a diagnosis of anorexia. An excess of physical exercise 4. NK cell killing 4. The oxidative burst refers to the process of generation of toxic products such as hydrogen peroxide. CD16) which bind to the constant region of IgG. Methanol .phenytoin Teaching Notes for Question 110 Theme: Antidotes For beta-blocker overdose. They have abundant Fc gamma receptors on their surface (FcgammaRIII. CTL-mediated lysis 2. Tricyclic antidepressant overdose may require intravenous neostigmine to counteract the 85 . a process termed antibody-dependent cell-mediated cytotoxicity (ADCC). Helper T-lymphocyte activation 3. Recruitment of neutrophils Teaching Notes for Question 109 Theme: Fas . fine lanugo hair. Beta-blocker .ethanol 5. Laxative abuse and self-induced vomitting may occur in the context of anorexia.

Northern blotting is used for the analysis of RNA not DNA. Other antidotes include desferrioxamine for iron. 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. which is concentrated to 20 times that of plasma. Discrete data may include number of children 86 . and vitamin K for warfarin. The uptake of iodide into the thyroid follicular cell is by active transport 2. dimercaprol for heavy metal poisoning.PEDIATRICS EXAMINATION REVIEW anticholinergic effects. 3. 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). ethanol for ethylene glycol. but further detailed analysis of the exact mutation will still be necessary to identify the gene responsible. dicobalt edetate for cyanide. Are found every 100 bases 2. and then the patterns of different fragment sizes compared from one sample to another. 2. These can be resolved according to size by gel electrophoresis. Are generated by digestion of DNA with bacterial enzymes 5. *TSH stimulates all steps in thyroid hormone synthesis . RFLPs can be used to link phenotype (disease) to genotype (sequence). 113-Which of the following statements are true: 1. Active uptake of iodide. Data may be described as qualitative or quantitative 2. Fuller's earth for paraquat. digoxinspecific antibody for digoxin. sequences not found in the bacterium's own DNA). About 99% of circulating T4 is protein bound 4. These enzymes are produces by bacteris (probably as a defence against viruses) and cut DNA at specific sequences. *T3 is 3 to 5 times more potent than T4.thyroid peroxidase catalyses the 2nd and 3rd steps. *Both T4 and T3 are highly bound to thyroid binding globulin or albumin. *T4 has a half-life of 7 days T3 is 1 day. which combines with tyrosine residues to give monoiodotyrosine (MIT) and di-iodotyrosine (DIT). When human DNA is subjected to digestion. 111-The following are true: 1. Coupling of 2 DIT residues gives T4 while addition of MIT and DIT gives T3. the DNA is cut only where the particular sequence occurs producing fragments of DNA. Oxidation of iodide to iodine. Are useful in diagnosis of multifactorial diseases 3. If a mutation changes the DNA sequence. Are useful in linked-gene diseases 4. calcium EDTA ± dimercaprol for lead poisoning. naloxone for opiates. 112-Restriction fragment length polymorphisms (RFLPs):1. N-acetylcysteine for paracetamol. the enzyme will not cut and so the sizes of the DNA fragments will be different. T3 is roughly 10 times more potent than T4 5. T4 has a half-life of 24 hours 3. and a beta-blocker for treatment of SVTs.

114-In an athymic child:1. Its' opening is associated with the first heart sound 5. These cells tend to be CD4 and CD8 negative. 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. The development of gamma/ delta -T-lymphocytes is unaffected 3. * Quantitative – which is data to which a certain number can be ascribed. It lies posterior to the sternum at the level of the fourth costal cartilage 3.g. T-cells play a vital role in macrophage function. The function of helper T-lymphocytes (Th) is affected while cytotoxic T-lymphocytes (Tc) activity is only moderately impaired 5. there is some evidence that they are able to develop in athymic mice possibly in the intestinal mucosa. Ordinal data does not include tumour staging 5. 115-The following statements are true about the mitral valve 1. T-helper cells activate macrophages by directly interacting with these cells via costimulatory molecules (CD40L. blood group type or ordinal (data can be ranked in some way) e. Discrete data may include serum glucose values 4. TNF-alpha.g. Macrophage function is impaired 2. number of children or continuous (assuming any value) e. Humoral responses are integrally dependent on T-cell help. The production of antibody is unaffected 4. It is bicuspid 2. plasma glucose. and therefore antibody production and B-cell function are affected when the thymus is absent.PEDIATRICS EXAMINATION REVIEW 3. This can be either discrete (only discrete values are assumed) e. tumour staging. 10% of intestinal T-cells express the gamma/ delta receptor. In humans. In DiGeorge. Patients with residual thymic tissue may have some T-cell function which tends to improve with age. Gamma/ delta T-cells in mice are found predominantly in epithelial tissues. This is due to the failure of development of the 3rd and 4th pharyngeal pouches. 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. Much of what is known about gamma/delta T-cells is derived from studies in mouse. the thymus fails to develop and is associated with parathyroid agenesis (hence tetany) and congenital defects of the heart and great vessels. Although these cells are thymus dependent. IFN-gamma .g. TNF-beta) and by secreting cytokines e.increasing macrophage antimicrobial activity. In humans <5%of T-cells express the gamma/delta T-cell receptor.g. The absence of thymus affects the development of both alpha/beta and gamma/delta T-cells. This can be either nominal (a naming scale) e. The associated papillary muscles are smaller than those of the tricuspid valve 4. many have no T-cell defects by adolescence. It can be regurgitant even if structurally normal 87 .g.

Angiotensin II promotes renal arteriolar dilatation 3. Sarcoidosis but not Amyloidosis is also a cause of lymphadenopathy. Rubella 2. Mitral regurgitation can occur with dilatation of the left ventricle. Herpes simplex type I. 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. even with a structurally normal valve. poxviral infections and Impetigo. Molluscum contagiosum Teaching Notes for Question 116 Theme: Vesicular Rash Other causes of vesicular rash in children include Varicella and Herpes zoster. 116-Causes of vesicular rash in children include:1. Closure of the mitral valve is associated with the first heart sound. Rheumatoid arthritis 5. Measles. Amyloidosis 3. which is converted to Angiotensin II by the Angiotensin Converting enzyme (ACE) in the lungs. Kawasaki disease 5. erythema infectiosum. scarlet fever. roseola infantum and other viral infections present with maculopapular rash 117-The following are causes of lymphadenopathy: 1. Hand Foot and Mouth disease 4. Rubella . Angiotensin II receptors are blocked by spironolactone 5. Roseola Infantum 2. Kawasaki disease. Angiotensin II inhibits aldosterone secretion by the adrenal glands 4. Opening is only audible in mitral stenosis (as the opening snap). SLE 4. Rheumatoid arthritis and thyrotoxicosis are all causes of lymphadenopathy. Thyrotoxicosis Teaching Notes for Question 117 Theme: Lymphadenopathy Rubella. Renin converts Angiotensin I to Angiotensin II in the kidney 2. Dermatitis herpetiformis 3.PEDIATRICS EXAMINATION REVIEW 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. 88 . These muscles are larger than those for the tricuspid valve as the LV pressure is higher. SLE. 118-The following statements are true for the Renin Angiotensin System:1.

In spite of all these. Cranial CT or MRI is essential with ophthalmology review. Less commonly. * Sinusitis. Abdominal pain 3. caused by 6th nerve palsy (abducens). * Iron deficiency anaemia. vitamin A and oral contraceptives). and ataxia. if a child is still symptomatic with progressive loss of vision. Whereas exotoxin is secreted by viable bacteria. Lumbar puncture will help to relieve headache and preserve the vision. Secondary causes include: * Drugs (corticosteroids. Headache is the commonest feature. If the pressure is high. paraesthaesia. It is a protein 2. some may have nausea.PEDIATRICS · · · · EXAMINATION REVIEW Angiotensin II promotes renal arteriolar constriction. Ataxia 5. Double vision 4. 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. * Polycythemia. Losartan is a competitive antagonist of the A II receptor. * Guillain-Barré syndrome. esp of the efferent arteriole. * SLE. * Adrenal insufficiency. dexamethasone can be given for two weeks or/and consider optic nerve fenestration or lumboperitoneal shunt. the papilloedema will progress and cause optic nerve atrophy. 119-Which of the following are common symptoms associated with raised intracranial pressure in children? 1. others include diplopia. 89 . It is idiopathic in 50%. * Head trauma. * Hyperadrenalism. * Hyperthyroidism. Acetazolamide can be started if patient cannot tolerate second or third lumbar puncture when symptoms recur. Papilloedema is a common early finding. neck stiffness. endotoxin is released only following bacterial cell lysis or death.the normal pressure lies between 12-16 mmHg. Unlike exotoxin. vomiting. 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. Morning headache 2. All endotoxin has the same physiological effect 5. 120-The following is true regarding endotoxin:1. * Hypoparathyroidism. tetracycline. Angiotensin II also stimulates aldosterone secretion leading to Na and water absorption. * Otitis media. it should be reduced by 40% . It is heat-stable 3. * Leukaemia. the effects of endotoxin are all non-specific. It is produced by some Gram-positive bacteria 4. Aldosterone receptors are blocked by Spironolactone Captopril inhibits ACE. If not treated.

puppies. Two or more fits within 30 minutes 90 . Hydatid disease may present to a surgeon as liver cysts. Important zoonoses include: Disease Animal source Campylobacter Cattle. pigs Q fever (Coxiella) Sheep. Usually blood transfusions are all that is needed. etc. cats Toxoplasmosis Cats Legionnaires’ disease (organism Legionella pneumophila) exists in water systems. goats Psittacosis Birds Hydatid disease Dogs. sheep. Poliomyelitis 5. Lyme disease 4. Teaching Notes for Question 122 Theme: Parvoviris B19 123-Definition of status epilepticus: 1. bats Toxocariasis Dogs. foxes.It may present with fever/ malaise/rash over face followed by rash over trunk. Can cause transient arthritis. Salmonellosis Teaching Notes for Question 121 Theme: Microbiology . In pregnancy it can cause fetal hydrops and fetal anemia. It is the causative agent for exanthem subitum 2. Legionnaires’ disease 2. 122-Regarding Parvovirus infections in children:1. sheep (Echinococcus granulosus) Leptospirosis (Weil’s) Rats Lyme disease Deer. cattle. puppies. protozoa. wild rodents (via tick bites) Rabies Dogs. Chronic anemia can be a manifestation in some children 4. poultry. Poliomyelitis is found only in humans. cattle. Arthritis is a very common manifestation in children 3.PEDIATRICS EXAMINATION REVIEW 121-The following are zoonoses: 1. Hydatid disease 3. Organisms may be bacteria. cattle.zoonotic infections. 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. In children with leukemia it can cause prolonged anemia. viruses. Aplastic crises due to parvovirus infection in sickle cell disease usually requires bone marrow transplant Parvovirus B19 infection may be asymptomatic or cause a condition called erythema infectiosum(Fifth disease/Slapped cheek disease). Infection in pregnancy can result in fetal anemia 5. jacuzzis etc. chlamydiae. cooling towers. A zoonosis is an infection that is naturally transmissible between vertebrate animals and man. kittens Salmonellosis Poultry. sheep Cryptosporidiosis Sheep.

Roll over by 2 months 2. Raised left atrial mean pressure. 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. Reduced cardiac output reduces renal perfusion thereby reducing glomerular filtration rate. Hypercapnia. 4. Kick a ball at 2 years 4. Riding a tricycle is expected at the 3 year . Reduced lung compliance. This milestone is at around 4 months. * As the seizures persist they often become localized. Ride a tricycle at 2 years 3. mouth twitching is a common sign of late status. Rapidly repeating tonic-clonic seizure with not returning to full consiousness Teaching Notes for Question 123 Theme: Epilepsy * Status epilepticus is defined as a condition in which continuous or rapidly repeating tonic-clonic seizures persist for 30 minutes or more. Pulmonary vascular resistance is increased due to raised left atrial pressures and chronic hypoxiemia 126-Evaluate the truth of the following descriptions of cytokines and their functional activities:- 91 . * It is often associated with incontinence but this is required for the diagnosis. 3. 125-The functional consequences of moderately severe mitral stenosis include: 1. A child should kick as well as jump at 2 years. Reduced pulmonary vascular resistance. Unresponsive patients who have been incontinent for 60 minutes 3. but there are many other diagnosis in a patient with mouth twitching and reduced GCS 124-Regarding Gross Motor Development. Not run until 2 years Teaching Notes for Question 124 Theme: Milestones Importantly. Mouth twitching lasting 60 minutes with reduced GCS 4. a normal child would be expected to:1. Reduced glomerular filtration rate. 5. Cruise by 1 year 5. 2. Cruising is moving around the round by holding on to furniture before independent walking begins and can be expected by 1 year. Tonic-clonic seizure lasting 30 minutes with incontinence 5.PEDIATRICS EXAMINATION REVIEW 2. a child won’t roll at 2 months as spuriously suggested by perpetrators in cases of non accidental injuries. Running is usually achieved by 18 months.

4.5 2. It also reduces expression of costimulatory molecules and MHC class II molecules on these 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. IL10 is produced by macrophages and some Th2 cells. IL-2 is produced by T-cells. Paradoxically IL-2 is also important in activation induced cell death (AICD). protein content of 500mg/day is within normal range 5. normal osmolality is 300-600 mosmol/kg 4. normal Na content is <20mmol/L 3. It is a growth factor for progenitor haematopoietic cells (all types). IL-6: induction of antibody secretion by plasma cells 5. Protein intake should be restricted in chronic renal failure. IL-3 : growth and differentiation of bone-marrow stem cells 2. In haemolytic uraemic syndrome. It is a potent inducer of IFN-gamma production by T-cells and NK cells. In acute renal failure.5mg/kg/hr 128-In children with renal failure 1. It also induces synthesis of acute phase proteins by hepatocytes and promotes myeloid stem cell differentiation. vascular endothelium and bonemarrow stromal cells. Grade V vesico-ureteric reflux spontaneously resolves in only 10% 92 . microangiopathic haemolytic anaemia is characteristic 5. It is an inhibitory cytokine . It stimulates the growth and activation of T-cells. normal pH is >5. 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. It stimulates the growth and cytolytic activity of NK cells and high concentrations can produce lymphokine-activated killer cells (LAK 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. Anaemia of chronic renal failure is due to low plasma ferritin levels 3. depending on the range of target cells: IL-3 is produced by T-cells.PEDIATRICS EXAMINATION REVIEW 1. IL-12: induction of IFN-gamma secretion by activated T-cells Teaching Notes for Question 126 Theme: Cytokines Cytokines often have many functions. It promotes differentiation of B-cells into plasma cells and increases antibody production.it inhibits macrophage production of IL-12. 127-Urine 1. IL-10: activation of B-cells 4. IL-12 is produced by macrophages and dendritic cells. T-cells. fibroblasts. dialysis is indicated with rapidly rising sodium 2.cells. IL-2 : activation induced cell death 3.3 2 true . and may act as a co-stimulatory signal for naive CD8+ T. NK cells and mast cells. IL-6 is produced by macrophages.

protein restriction is usually inappropriate 129-Lung surfactant 1.IG is recommended for infants less than 2 yr with chronic lung disease or prematurity but should not be given to those with CCHD because of increased complications. Lymphopenia is common. involving anaemia. 3. Scattered areas of atelectasis may be seen in approx 30%. It is linked to maternal smoking. hypertension. and uraemia. The WBC count and differentials are usually within normal limits in bronchiolitis. 40% resolve spontaneously. acidosis. 5. CXR commonly shows hyperinflation. 4. It causes an increase in lung compliance only (not chest wall compliance). Gastric distension increased motility 93 .CXR commonly reveals hyperinflation. the following are true :1. In children with CRF since growth failure may occur and the metabolic demand is high. thrombocytaemia. But there is no convincing evidence to support its use. Somatostatin reduces gastric motility 2. drug OD. Decreases the surface tension within an alveolus 2. and rising Cr are all indications (if they are unresponsive to treatment) Erythropoitin is made in the kidneys. Ribavarin is curative. which stimulates red cell production. Is a glycoprotein 4. an antiviral drug may be given to infants with cong heart disease or BPD. RSV. It is produced by type-II pneumocytes and is seen at about 24 weeks gestation. RSV immune globulin is recommended in infants with cyanotic congenital heart disease to prevent severe RSV infection. Causes an increase in chest wall compliance 3. Teaching Notes for Question 130 Theme: Bronchiolitis Infants whose mothers smoke are more likely to get bronchiolitis than those of non smoking mothers. Ribavarin. Maintains the same surface tension for different sized alveoli 5. 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. 2. This helps to minimise uraemia Usually seen post diarrhoea (especially E Coli 0157). which prevents alveolar collapse by reducing alveoli surface tension. 130-With regards to bronchiolitis. It is given IV/ IM just prior to and during RSV season.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 128 Theme: Renal failure Rising K+. 131-The following concerning gastrointestinal hormones are true 1. grades I-III 85%.

Epstein Barr virus 2. Lower than intracellular pH. Glucagons increases GI motility 4. Normal pH absolutely essential for enzyme function/ion distribution/protein stability. pH increases if anything because of excretion of bicarbonate. pH measured in pH probe. 4. 3.46 nmoles/L.they use this question a lot. however the following 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 exocrine 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 and action of gut hormones Glucagon (pancreas) gluconeogenesis and reduces GI motiltiy 132-Normal arterial pH is: 1.35 . In pregnancy. 2. Measured rather than calculated in a pH probe. Intracellular pH = extracellular pH . Haemophilus influenzae 5. 3.PEDIATRICS EXAMINATION REVIEW 3. Equal to [H+] of 34 . 2. 5. It is a purulent conjunctivitis. 133-The following organisms cause conjunctivitis:1. which develops 5-14 days after birth and is indistinguishable from gonococcal infection. 5. Teaching Notes for Question 132 Theme: Acid/base balance 1. Secretin inhibits pancreatic bicarbonate secretion 5.7. Adenovirus 4. 4. Chlamydia trachomatis 3. 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. Not essential to maintain ion distribution.45 = [H+] 36 – 45 nmol/L. pH 7. It is diagnosed on a swab scraped over the 94 . Pancreatic polypeptide inhibits pancreatic secretion Teaching Notes for Question 131 Theme: GI hormones The roles of individual gut hormones are not completely defined. Decreased in pregnancy.

thalassemia major) causes severe transfusion-dependent anemia with high reticulocyte count. There are 18 bronchopulmonary segments 3. cardiac dysfunction) o The physical findings are related to severe anemia. The vagus nerve is responsible for normal bronchial tone 2. In the homozygous state. and spleen. There are 5 lobes divided into 18 segments. Diagnosis is by haemoglobin electrophoresis. 3. The anaemia becomes manifest in late infancy. gall bladder. o The skull and other bones may be deformed secondary to erythroid hyperplasia with intramedullary expansion and cortical bone thinning. Hepatomegaly related to significant extramedullary hematopoiesis typically is observed. At birth there are 25 million alveoli. This overload causes clinical problems similar to those observed with primary hemachromatosis (eg. coxsackie and herpes simplex are other viral causes. beta thalassemia (ie.PEDIATRICS EXAMINATION REVIEW lower eyelid (to allow cells to be collected – don’t forget it is an intracellular organism) by direct fluorescent antibody. The surface markings of the right middle lobe approximate to the axilla 5. Most of the alveoli in the adult lung are present at birth 4. o Skin may show pallor from anemia and jaundice from hyperbilirubinemia. o Abdominal examination may reveal changes in the liver. liver dysfunction. Anaemic patients should receive regular iron supplementation 5. ELISA or PCR. Increased iron deposition resulting from multiple life-long transfusions and enhanced iron absorption results in secondary iron overload. 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. 134-The following statements are true: 1. enterovirus. Diagnosis can be made by Haemoglobin electrophoresis 4. related to either severe anemia or iron overload. and iron overload resulting from transfusion and increased iron absorption. 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. Gonococcal conjunctivitis presents earlier than chlamydial disease (usually within 2 days). 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. The major component of surfactant is dipalmitoyl lecithin Teaching Notes for Question 134 Theme: Anatomy 2. which increase in the adult to 300 million per lung. Don’t forget sexual health screening for the mother and informing public health of ophthalmia neonatorum. 4. Beta thalassaemia major presents at birth with profound anaemia 2. Adenovirus causes conjunctivitis in summer outbreaks. is diagnosed on gram stain and culture and should be treated with IV penicillin and chloramphenicol eye drops. The reticulocyte count is low 3. 95 . endocrine dysfunction. extramedullary hematopoiesis. ineffective erythropoiesis. o Heart examination may reveal findings of cardiac failure and arrhythmia.

usually benign and arising from the meninges. testes. and soft tissue pool. which metastasise to the brain. Pituitary tumours are around 5% of the total. Haemodialyisis effectively eliminates lead 4. Cerebral metastases comprise around 70% of all brain tumours. and thyroid. Is associated with basophilic stippling in white cells Teaching Notes for Question 137 Theme: Lead poisoning Unlike almost all other trace metals blood rather than urine concentrations are the most useful for assessment of lead exposure. 5. Urinary lead concentration is the most useful indicator of exposure 2. 15 . Lead poisoning is associated with basophilic stippling of red blood cells. breast carcinoma and malignant melanoma. the commonest being astrocytomas. The most frequent (around 40 . Chelation removes lead from the soft tissue pool. There are 2 bone pools a shallow and a deep in addition to the blood. The gall bladder may contain bilirubin stones formed as a result of the patient's life-long hemolytic state. Haemodialysis is useless at eliminating lead because of the large non-blood pools. 2. These are of variable malignancy . o Extremities may demonstrate skin ulceration. usually multiple. 4. Meningioma is usually benign. Gastric carcinoma frequently metastasises to the brain.25% are brain secondaries. transfusion-associated viral hepatitis resulting in cirrhosis or portal hypertension also may be seen.PEDIATRICS EXAMINATION REVIEW Patients who have received blood transfusions may have hepatomegaly or chronic hepatitis due to iron overload. 3. especially affecting the pancreas. Astrocytomas arise from the neuroglia. 75% of lead in children is in the bone.50%) arise from the glial cells. Asymptomatic children with lead levels >46µg/dL are treated with courses of DMSA (dimercaptosuccinic acid) for 2 weeks followed by a 2 week interval to assess post-treatment rebound which occurs due to equilibration from bone stores. 138-Benign rolandic epilepsy :1. In asymptomatic children with increased lead levels DMSA therapy is recommended 3.20% are meningiomas.glioblastoma multiforme is the most malignant subtype. Teaching Notes for Question 136 Theme: Central nervous system Around 80% of brain tumours are primary. The most frequent tumours. The deep bone pool has a half life of decades. 137-Regarding lead poisoning:1. Splenomegaly typically is observed as part of the extramedullary hematopoiesis or as a hypertrophic response related to the extravascular hemolysis. Glioblastoma multiforme is a benign neoplasm. are bronchial carcinoma. o Iron overload also may cause endocrine dysfunction. Is a common form of partial epilepsy 96 . Around 15 . 136-The following statements concerning CNS tumours are true: 1. The half-life of the deep bone compartment is months 5.

At extremely high concentrations e.the most common partial epilepsy in childhood.g. At higher concentrations. 3. The thoracic duct is only present on the left side. activated T-cells. Involves seizures that often occur at school 4. Treatment with carbamazepine. 2. A prominent tubercle on the inner border of its upper surface marks the 97 . TNF acts as an endogenous pyrogen by increasing energy mobilisation in fat and muscles. may not require treatment. activated NK-cells and occassionally mast cells. The first rib is flattened from above downwards. Seizures usually occur in sleep: sensory aura followed by GTC seizures often involving face. Induction of killing by cytotoxic T lymphocytes (CTL) Teaching Notes for Question139 Theme: Tumour necrosis factor (TNF) TNF-alpha is produced by mononuclear phagocytes. It activates phagocytes. Teaching Notes for Question 140 Theme: Head-Neck Stellate ganglion is formed by the c8-t1 nerves. Stellate ganglion. decreased myocardial contractility and intravascular thrombosis. Dendritic cell migration and maturation is enhanced. Resetting of the temperature threshold in the hypothalamus resulting in pyrexia 5. It is the shortest flattest and most curved of the ribs. Disseminated intravascular coagulation in systemic Gram-negative bacterial infection 4. EEG shows typical cento-temporal spikes. Production of acute phase proteins by hepatocytes 2. It has a wide range of systemic effects. in gram-negative bacterial sepsis. Mobilisation of B-cells from the bone marrow 3. TNF causes circulatory collapse associated with vasodilatation. It is a procoagulant. Excellent prognosis.PEDIATRICS EXAMINATION REVIEW 2. arms. 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. 140-The neck of the 1st rib is related to: 1. Has a poor prognosis 3. It also produces cachexia by inducing appetite suppression. 139-Which of the following biological responses or consequences is mediated by the release of tumour necrosis factor (TNF)–alpha? 1. 15-20% of childhood epilepsy. The thoracic duct on the right. Phrenic nerve. Seizures usually stop in mid teens. Has a typical EEG with a 3 per second spike and wave pattern Teaching Notes for Question 138 Theme: Epilepsy Benign rolandic epilepsy. Can be successfully treated with carbamazepine 5. and chronic production suppresses bone marrow haematopoiesis. Scalenus anterior. 5. enhancing cytokine production and microbicidal activity. The superior intercostal artery. 4. and increases synthesis of acute phase proteins.

Spinothalamic tract degeneration above the section level 5. Loss of motor function 2. Is related to Sandifer Syndrome 5. 141-Which of the following would cause an increase in the carbon monoxide transfer factor? 1. 142-A patient develops low back pain which is progressive. THE SUPERIOR INTERCOSTAL ARTERY 3.PEDIATRICS EXAMINATION REVIEW insertion of scalenus anterior. Anaemia Teaching Notes for Question 141 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. He is found to a spinal schwannoma. Alveolar inflammation. Is related to Rett syndrome 98 . as this is provided by the ganglia. 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. Which of the following is likely to result from this? 1. temperature and preservation of light touch 4. which makes it different from peripheral neuropathy which is not dermatomal. It is resected. and usually not entirely sensory or motor. Exercise increases pulmonary blood flow. Churg-Strauss 3. 143-Autism :1. Exercise 5. Extra-pulmonary restriction 2. Can be treated successfully with psychotherapy 2. A BRANCH OF T1 THAT CONTRIBUTES TO THE BRACHIAL PLEXUS. Extra-pulmonary restriction would increase the density of blood per unit lung volume with more red cells coming into contact with the CO. There is no effect on the sympathetic supply. Is sometimes related to poor parenting 3. Loss of tendon reflex 3. THE SYMPATHETIC TRUNK 2. but division of the posterior spinal root occurs. ( and ASTHMA). Is related to Fragile X syndrome 4. Goodpasture’s syndrome 4. Structures crossing the neck of the first rib from medial to lateral are: 1. Posterior division causes spinothalamic tract degeneration. Loss of pain. Alveolar haemorrhage.

Patients with asplenia or hyposplenia (sickle cell disease. NOT related to parenting methods. protodeclarative pointing. immunological theories. abnormal use of speech. The white pulp forms a sleeve around penetrating arterioles and contains numerous Tcells. child abuse or neglect. It is comprised of the red pulp. which contains an abundance of erythrocytes. For Penicillin-allergic patients. Inability to recognise or appreciate the feelings of others and respond to social cues. 4. Co-Trimoxazole may be an alternative for children. The onset of symptoms may be extremely rapid. Following splenectomy. Rett’s syndrome: occurs in girls. 2. Macrophages located in the marginal zones of the spleen are very efficient at trapping and retaining polysaccharide antigens. 99 . The lymphoid function is carried out in the white pulp. handflapping. ritualistic behaviour. Usually diagnosed around 2-3 years of age as that is when speech delay becomes readily apparent.echolalia. The marginal zone and PALS are populated by interdigitating dendritic cells specialized in the presentation of antigen to T-cells. but is not recommended in adults. Following splenectomy. 3. Imagination: restricted range of interests. Social interaction: ‘autistic aloneness’. An area called the marginal zone is closely associated with this periarteriolar lymphoid sheath (PALS) and is rich in B-cell follicles. Asperger’s describes those children who have impairments in the core features without learning difficulties. More common in boys than girls. All patients regardless of underlying condition. 144-With regards the spleen: 1. Amoxycillin 500 mg od. Inflexible routines. Treatment of developmental disorders: symptomatic treatment as causes poorly defined. communication and imagination. The risk does not diminish with time post-splenectomy. 5. especially language and manual dexterity skills. These are then presented to B-cells and an appropriate immune response is initiated. erythromycin should be used (250 mg bd adult dose – 10 mg/kg child).PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 143 Theme: Autism and pervasive developmental disorders Features impairments in socialization. Initially normal development with regression and loss of skills before 2 years of age. The spleen is involved in the destruction of encapsulated organisms. rocking. Unable to modify their behaviour on the basis of these social cues. autism is a feature of fragile X). Late development of social smile. some patients with coeliac disease) are at increased risk of overwhelming bacterial infection. and death may occur in hours. Educational and behavioural methods are mainstay of treatment. Communication: verbal and non-verbal language affected. Children's dosage is 10mg/kg. low dose penicillin & pneumovax are recommended. especially those with congenital asplenia. They tend to be diagnosed later because they are able to cope and adapt better than severe autistics. Autistic features. use of gestures. infective aetiology. Inability to adapt to change. It is highest in those who have had a splenectomy for lymphoproliferative disease (NHL. Basophilic stippling of neutrophils is seen with asplenia. should be on life-long antibiotic prophylaxis . Causes: genetic (rate of autism in siblings increased. CLL) or who have concomitant liver disease. Adult doses: Penicillin V 250 mg bd. it is largely the kupffer cells in the liver that filter micro-organisms. Poor speech development. Teaching Notes for Question 144 Theme: Spleen The spleen is specialized to trap and mount an immune response against blood borne antigens. Poor development of joint attention.either Penicillin V bd or Amoxycillin od. Parents often report child not wanting to be cuddled as an infant. Poor development of facial expression. Stereotypies eg head banging. Poor symbolic or imaginative play. Gaze avoidance. Asperger’s syndrome: actually modern view suggests that Asperger’s describes the milder autistic children ie an autistic spectrum of disorders exists. with a preference for Pencillin V.

and splenomegaly is seen in 50% at diagnosis. Leukotriene inhibitors are associated with the development of Churg-Strauss . All other patients should receive the 3 vaccines even after splenectomy. by definition bronchial hyper-responsiveness must be present. The eosinophils to the lung are recruited from the bone marrow 2. Patients are susceptible to infection with bacteria and viruses 3. Teaching Notes for Question 146 Theme: Asthma. It is often a slowly progressive disease and should not be treated in the early stages. 145-Chronic lymphocytic leukaemia :1. after a blood culture (if possible).protein conjugate (either tetanus or diphtheria conjugates) and meningococcal A & C polysaccharide vaccines at least one week before surgery. Blocking IL-5 will therefore be therapeutic. 147-With regards severe combined immunodeficiency:1. 5. Booster immunization may be required if levels of antibody fall below protective levels. liver and lymph nodes. Basophillic stippling is seen in lead poisoning. as there may be some benefit. Is asymptomatic at diagnosis in less than 10% of patients 3. Patients travelling to malarial areas and areas where penicillinresistant pneumococci have been found require specialist advice. Patients have defective T-cell dependent antibody responses and defective cell-mediated immunity 2. 146-In asthma:1. 3 weeks after and then at yearly intervals thereafter. All adult patients should receive an annual influenza immunization. Has a median survival of less than 5 years 5. Blocking interleukin-5 maybe therapeutic 4. It is asymptomatic at diagnosis in over 30% of patients. Blood levels of antibodies to these polysaccharides should be measured before. Howell Joly bodies are seen in asplenia. bone marrow. IL-5 is produced by T helper cells (TH-2) and recruits eosinophils from the marow. Annual hospital follow-up is recommended. Is associated with smear or smudge cells in the peripheral blood 2. haemophilus influenzae type B capsular polysaccharide . Median survival is 3-5 years. should be started immediately. Leukotriene inhibitors are associated with Churg-Strauss syndrome and causality can not be excluded. X-linked SCID is caused by a defect in the IL-2 receptor 100 . 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. Steroids are contra-indicated if the patient is a diabetic 3. Bronchial hyper-responsiveness (BHR) does not have to be present. spleen. Any asplenic/hyposplenic patient who develops a sudden febrile illness should be treated promptly with full dose antibiotics.PEDIATRICS EXAMINATION REVIEW Patients undergoing elective splenectomy should receive pneumovax. Smear or smudge cells are often seen in the peripheral blood which represent the fragile malignant cells which have been squashed as the blood film is spread. Intravenous penicillin. but one third of patients die of causes other than their CLL. Should be treated aggressively in the early stages of the disease 4. pathophysiology In asthma.

all of which are autosomal recessive. B-cells are present (T-B+ SCID). Plays in parallel with his/her peers 4. 9 and 15). Cisapride 2. Humoral immunodeficiency occurs because of a lack of T-cell help. Should imitate observed actions 5. 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 immunodeficiency Patients with SCID always have deficient T-cells but may or may not have absent B-cells. 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. Progesterone 3. ADA and PNP are enzymes which catalyze the metabolism of purines via the uric acid pathway.PEDIATRICS EXAMINATION REVIEW 4. 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. Several defects can lead to the phenotype of severe combined immunodeficiency.normal developmental milestones 101 . 149-A normally developing 18-month-old child: 1. 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. In Xlinked SCID there is a mutation in the gene encoding the common gamma chain – a chain shared by receptors for a number of interleukin cytokines -(IL-2. Other known causes of T-B. purine nucleoside phosphorylase (PNP) deficiency. In this type of SCID. There is thus impaired T-cell differentiation as the interleukins are important T-cell growth factors.and Tlymphocytes.SCIDs are due to adenosine deaminase (ADA) deficiency. 148-Lower esophageal sphincter tone is decreased by: 1. Deficiency of either of these enzymes leads to the accumulation of toxic metabolites which severely affect developing T-cells.and B-cells. Patients with SCID are susceptible to infection with bacteria and viruses as there is a complete defect in adaptive immunity. T-B. Functional Rag proteins are required for initiation of recombinatorial rearrangement of the genes encoding the antigen-specific receptors of both T. Suxamethonium 4. Ganglion blockers 5. Should be able to copy a vertical line Teaching Notes for Question 149 Theme: Developmental milestones 18-month-old child . 4.SCID include mutations of DNA-dependent protein kinase (DNA-PK . 7. Should recognise his/her name 2. Can easily distinguish primary colours by name 3. or RAG-1 or –2 deficiency. B-cell development is also affected to a lesser extent. Patients with mutations resulting in non-functional Rag fail to develop both B.an enzyme involved in DNA repair) and lack of expression of MHC class II or MHC class I molecules (bare lymphocyte syndrome).

These include the rash of HSP. ASD’s account for 40% of all CHD 3.General understanding * points to own or doll’s nose. a vasculitis that can cause GI bleeding. such as Marfan’s or Noonan’s. 80% of children with Down’s Syndrome have CHD 5.some children are not walking yet. eyes on request . ASD (secundum) is the second most common defect and accounts for 6-8% of all lesions. Approximately 3% of patients with congenital heart disease have an identifiable gene defect. 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.Speech and language * 6-20 recognisable words * names 1-2 simple objects . Approx.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. Webbed neck and low hairline 4.PEDIATRICS EXAMINATION REVIEW Gross motor development: * walks well with feet only slightly apart . 40% of patients with Turner’s Syndrome have coarctation and-or aortic stenosis. 2. VSD’s account for 70% of all CHD. hair. 151-The following are clues to the cause of an upper gastrointestinal (GI) bleed: 1. Approx. Perianal skin tags 5. 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: . Teaching Notes for Question 150 Theme: Congenital heart disease VSDs account for 25% of all congenital heart disease and are the most common anomaly. Hypopigmented spots on the lips suggestive of Peutz-Jeghers syndrome 3. Approx. 13% of patients with a chromosomal defect have associated CHD – heart disease is found in over 90% of patients with trisomy 18.2-3 parts of the body * imitative play * points to named pictures . Purpuric rash suggestive of Henoch-Schonlein purpura (HSP) 2. 13% of neonates with chromosomal abnormalities have associated CHD 4. Overall. 102 . 50% of patients with trisomy 21 and 40% of those with Turner’s syndrome.Social behaviour * feeding .

Acromegaly 3. Ipsilateral ptosis 2. Gestational diabetes 103 . Levator veli palatini 4. Heterochromia refers to different pigmentation of the iris compared to the normal eye and is a feature of congenital Horner’s. Palatopharyngeus Teaching Notes for Question 152 Theme: Soft palate Hyoglossus is an extrinsic muscle of the tongue. 152-The muscles of the soft palate are: 1. Contralateral mydriasis 3. Partial gastrectomy 4. The affected eyeball often seems sunken (enophthalmos) owing to slight elevation of the lower eyelid. infarction b) Cervical cord: syringomyelia. Tensor veli palatini 2. demyelination c) T1: apical carcinoma of lung (Pancoast syndrome). A webbed neck and low hairline would indicate Turner Syndrome which is associated with GI vascular malformations. jugular foramen syndromes All the features of Horner’s are ipsilateral. Causes depend on the level of the lesion: a) Brainstem: tumour. Exophthalmos Teaching Notes for Question 153 Theme: Features of Horner's syndrome Horner’s syndrome consists of ptosis. 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. Wilson's disease 5. demyelination. neck surgery e) Internal carotid: dissection. 153-Horner’s syndrome invariably produces :1.PEDIATRICS EXAMINATION REVIEW Peutz-Jeghers syndrome . trauma d) Cervical sympathetic chain:thyroid carcinoma.a syndrome manifesting with multiple intestinal polyps is associated with hyperpigmented spots on the lips. cervical ribs. carotid body tumour. Loss of sweating over the ipsilateral face 4. Heterochromia 5. Hyoglossus 5. Haematochezia suggests a lower GI bleed. Malabsorption 2. Perianal skin tags are found in inflammatory bowel disease. tumour. miosis and anhydrosis and is caused by interruption of the sympathetic supply to the eye. Palatoglossus 3. 154-An abnormal response to oral glucose loading is seen in:1. Loss of sweating may not occur if the lesion is distal to the superior sympathetic ganglion.

155-With regard to the visual pathways: 1. Chronic hypoxia due to cyanotic conditions 104 . or lower quadrantinopia if in the parietal region. 156-The O2-Hb dissociation curve is displaced to the right in: 1. Fetal Hb 2. A lesion of the optic tract/radiation or occipital cortex will result in a contralateral homonymous hemianopia. An elevated glucose 2 hours after loading suggests diabetes. In Wilson’s disease there is an exaggerated rise in GH. Chiasmal lesions (e. Methemoglobinemia 3.g. 5. therefore.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. Partial gastrectomy produces a lag response. Ventilation is reflexly depressed by metabolic alkalosis. there is a paradoxical rise in GH levels. Increased lactic acid production 4. The pupillary light reflex is preserved in lesions of the optic nerve 4. A lesion of the optic chiasma will cause a homonymous hemianopia 2. Metabolic alkalosis is the reduction in H+ concentration by any means other than decreased PCO2. Hypoventilation is induced by metabolic alkalosis to raise PCO2 and retain acid in order to bring pH back into the normal range. 3. In Acromegaly. Increased central chemoreceptor firing rates decrease ventilation rates. 2. 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. be abnormal in lesions of the optic nerve. in addition there will be enlargement of the blind spot. 4. Teaching Notes for Question 154 Theme: Metabolic alkalosis Increased peripheral chemoreceptor firing rates (but not central) increases ventilation rates. a lesion of the optic radiation with result in a contralateral upper quandrantinopia if in the temporal region. 155-Metabolic influences on pH 2: Which of the following statements are true? 1. The light reflex gives an indication of optic nerve function and will. If incomplete. A lesion of the left occipital lobe will result in a right homonymous hemianopia 3. Lesions of the optic nerve cause an enlargement of the blind spot 5. Hyperventilation induced by metabolic alkalosis returns arterial pH to the normal pre-set points. Metabolic alkalosis can be induced by vomiting from the stomach. due to compression by pituitary tumors) result in bitemporal hemianopia.

1-0. IVIG and steroids can be used to treat ITP. Renal blood flow = plasma flow/(1 . First line treatment is imipramine 105 . Metachromatic leukodystrophy Teaching Notes for Question 157 Theme: Intravenous immunoglobulin (IVIG). Normal filtration fraction is 0. IgA deficiency 3. Human normal immunoglobulin is prepared from pools of at least a thousand donations of human plasma and contains antibody to common viruses. Guillain-Barre syndrome 2. 5% of 5 year old children are affected 3. High dose IVIG (2g/kg) is used to treat Kawasaki disease. It can be given IM for protection of susceptible contacts against hepatitis A. GFR can be measured using Doppler ultrasound 5. where there is an increase in 2.3 DPG Teaching Notes for Question 156 Theme: Oxygen hemoglobin dissociation curve When shifted to the right.HCT) 4.2 3. In a 3 year old investigations must be carried out 2. O2 is easily given up and this will be beneficial in conditions when the body is working hard. Intravenously. * Increase in CO2 production due to increased metabolism * Increase in H+ * Hyperthermia * Chronic hypoxic states/anemia. Urine dipstick is a useful investigation 4. For the same reason. Kawasaki disease 5.PEDIATRICS EXAMINATION REVIEW 5. IVIG is now preferred to plasma exchange in the treatment of GuillainBarre syndrome.3 DPG 157-Intravenous immunoglobulin (IVIG) is used in the treatment of:1. 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. Metachromatic leukodystrophy is an autosomal recessive lysosomal storage disorder. Increased 2. Idiopathic thrombocytopenia purpura 4. 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. blood should be given with caution: transfusion reactions are more likely. measles and rubella. 158-In the kidney 1. The filtration fraction is the ratio of GFR to renal blood flow 2.

include altered consciousness. They are focal frontotemporal seizures. Diarrhoea can complicate diabetes mellitus due to the autonomic neuropathy. However complex partial epilepsy can involve generalised seizures such as absence and tonic-clonic seizures.PEDIATRICS EXAMINATION REVIEW 5. Consciousness is altered 3. Boys more common than girls. Hyperventilation provokes absence seizures. 160-Endocrine disorders that result in diarrhoea include:1. Diagnosis is by urinary 5HIAA.when child has never been dry. Right sided cardiac valve lesions can also occur. There is an increased incidence of osteosarcoma 106 . resection of the primary or octreotide to reduce symptoms. Carcinoid results in diarrhoea and facial flushing once metastasis to the liver has occurred. Night time wetting more common than daytime wetting. Primary hyperaldosteronism 5. Conditioning is usually successful Teaching Notes for Question 159 Theme: Enuresis Enuresis: is the involuntary voiding of urine by day and/or night. not complex partial seizures. IDDM or neurological disorder. Treatment is by embolisation of the secondaries. Cushing’s syndrome 2. Hyperparathyroidism 3. Ethosuximide is an affective treatment Teaching Notes for Question 161 Theme: Complex partial seizures Complex partial seizures. The tumours secrete 5 HT. Medication: tricyclic antidepressants. star charts. Must rule out organic causes first eg UTI. Thyrotoxicosis 4. EEG changes disappear in sleep 5. Ethosuximide is only effective in absence seizures. Sleep often enhances EEG abnormalities. desmopressin-tablets or nasal spray. and are commonly sited in the terminal ileum or appendix. by definition. dermatitis and dementia. Genetic factors involved.bell and pad. Mainstay is behavioural conditioning methods. Carcinoids make up 10% of all small bowel tumours. Carcinoid syndrome Teaching Notes for Question 160 Theme: Endocrine causes of diarrhoea Addison’s disease and thyrotoxicosis can both present as diarrhoea. Developmental delay in neurological maturation. 162-In osteogenesis imperfecta 1. Absence seizures and generalised tonic-clonic seizures may occur 2. Primary enuresis. The diversion of typtophan from the niacin synthesis pathway can result in a pellagra à diarrhoea. 161-Regarding complex partial epilepsy:1. Carbamazepine and sodium valproate are effective treatments for complex partial seizures. Hyperventilation often provokes a complex partial seizure 4. Occurs in 10-15% of 5 year olds and 3-5% of 10 year olds. Treatment: simple methods such as reducing fluid intake prior to going to bed. histamine and bradykinins.

In the tetralogy of Fallot. 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.The majority of defects are in the membranous septum. bisphosphonates have been shown in series to improve BMD and reduce fractures. and is associated with pulmonary stenosis. Is metabolized by plasma esterases 5. Hearing abnormalities are common 5. Has an oral bioavailability of 50% 4. Although not a licenced treatment. Prolongs the duration of action of suxamethonium 3. and anterior to the septal leaflet of the tricuspid valve. RV and LV pressures equalise. Diagnosis is usually made by bone biopsy Teaching Notes for Question 162 Theme: Osteogenesis imperfecta OI results from mutation in Type I procollagen gene. the murmur may be inaudible during the first few days of life as the pulmonary pressures are high. A restrictive VSD (<0. poor growth. Small VSDs are usually asymptomatic with a harsh pansystolic murmur at the LLSE – in neonates. the defect lies between the crista supraventricularis and the papillary muscle of the conus. profuse sweating. posteroinferiorly. recurrent chest infections and cardiac failure in infancy. Tricuspid atresia is a recognised association 5. May regress by incorporation of the tricuspid leaflet tissue 4. Blue sclera is also found in Marfan’s. Can present with heart failure in the neonatal period 2. 164-Neostigmine: 1. the size of the left-to-right shunt increases and clinical symptoms become apparent – dyspnoea. feeding difficulties. OI is a fairly easy clinical diagnosis to make. Pamidronate is a recognised treatment in children with this condition 4.5 cm2) is usually associated with normal RV pressures. Crosses the placenta Teaching Notes for Question 164 Theme: Anticholinesterases * Neostigmine also inhibits pseudocholinesterase and so can prolong the duration of action of suxamethonium. Has been used in the treatment of SVT 2. As the PVR continues to fall in the first few weeks after birth.PEDIATRICS EXAMINATION REVIEW 2. 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. 163-Ventricular septal defects: 1. Ehlers-Danlos and normal infants. biopsy is rarely done. 107 . Blue sclera is diagnostic 3. 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. Results in wide splitting of the second heart sound 3. In nonrestrictive VSDs (>1 cm2).

The list of systemic associations with cataracts is too long to discuss in full. Molecules consist of 21 carbon atoms. systemic diseases A cataract is simply any loss of the transparency of the lens of the eye. 166-The following statements are true:1. Atopy. Down’s syndrome. The Babinski response is usually extensor until the infant begins to walk (usually from 1 year). Wilson’s disease. Exceptions are the plantar reflex (9 months).2%.PEDIATRICS EXAMINATION REVIEW * Its oral bioavailability is only 1 . Toxoplasmosis and CMV. asymmetric tonic neck reflex (9 months). A newborn baby from 32 weeks gestation with a normally functioning visual cortex will turn the head towards a light. 108 . and usually flexion of the thighs at the hips. but important associations include: * * * * * * * * * * Myotonic dystrophy (not muscular dystrophy!). Relapsing polychondritis 5. 165-Recognized associations of systemic conditions and cataracts include: 1. stepping reflex (11 months). but also approx 30% in the bile. Fabry’s disease. Deep tendon reflexes are present and usually brisk at birth. Cause retention of sodium. 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. A moro reflex with the fists remaining clenched is normal. 3. it does not cross the BBB or the placenta owing to its highly ionized state at physiological pH and also its quaternary amine structure. Diabetes mellitus. Muscular dystrophy 2. Galactosemia 3. 2. 2. Deep tendon reflexes are difficult to elicit at birth 4. An extensor Babinski response is normal in the first year 5. Hyperthyroidism 4. * It is metabolized by plasma and hepatic esterases and is excreted predominantly in the urine. The moro reflex consists of arm extension followed by flexion with extension of the fingers. Are secreted from the zona fasciculata within the adrenal cortex. Congenital rubella. Galactosemia. Septicemia Teaching Notes for Question 165 Theme: Cataracts. The plantar grasp persists longer than the palmar grasp 3. A moro reflex with the fists remaining clenched is abnormal. 167-Glucocorticoids: 1.

Teaching Notes for Question 169 Theme: Thyroid physiology TRH is secreted by the hypothalamus. Iodine is taken up passively by the thyroid gland. which in turn up-regulates synthesis of hormones by the thyroid gland. thrombocytopenia. coupled with sodium. There is a slight mineralocorticoid effect. T4 is released bound to thryroglobulin. Behavioural modification. Batten's disease 4. 4. abdominal pain. T4 is released in greater quantities than T3. 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 medicationmethylphenidate (Ritalin). It stimulates release of TSH from the anterior pituitary. Thyroid peroxidase catalyses the formation of thyroxine. Methylphenidate (Ritalin) should not be given later than 4pm 4. Iodine is taken up by secondary active transport. 2. parenting techniques. 168-In the treatment of attention deficit hyperactivity disorder :1. TRH is released by the anterior pituitary. Pharmacological intervention includes the use of stimulant drugs such as amphetamines 3. Parenting techniques can be taught 5. which are cleaved from the parent molecule prior to release.PEDIATRICS EXAMINATION REVIEW 4. and also an antiinflammatory effect. Not to be taken after 4pm. Behavioural therapy is never useful 2. dexamphetamine. Iodination of the tyrosine residues of thyroglobulin leads to the production of T3 and T4. Congenital adrenal hyperplasia 109 . Side effects include difficulty sleeping. The main effects are in protein. headache. Inhibit gluconeogenesis. 5. Primary ciliary dyskinesia 3. 169-Concerning the synthesis of thyroid hormones: 1. Subsequently it is oxidised and coupled to thyroglobulin. loss of appetite. Outcome: symptoms tend to lessen as child gets older. 170-The following diseases are inherited in an autosomal recessive manner:1. Teaching Notes for Question 167 Theme: Glucocorticoids The glucocorticoids consist of cortisol and corticosterone. 3. Insulin dependant diabetes 2. Reduce lymphocyte counts. glucose and fat metabolism. 5.

Affected children suffer progressive mental impairment. Teaching Notes for Question 171 Theme: Catecholamines 172-In a healthy baby the transition from fetal to neonatal circulation involves:1. 173-The pancreas: 1. Is entirely retroperitoneal 110 . The umbilical artery is a branch of the internal iliac artery. and hence may cause a reflex bradycardia. Prostaglandin E2 keeps the ductus open. though it does have some action at the beta-adrenoceptor. dynein. Increased oxygen concentrations 2. blood flow in the ductus is reversed ie from left to right. The ductus arteriosus closes in response to decreased oxygen concentrations 5. Blood flow in the ductus arteriosus continues from right to left until its closure 3.Batten Disease is an AR disorder also known as Spielmeyer-VogtSjogren-Batten Disease. Drop in pulmonary artery pressures N. Is a neurotransmitter at the pre-ganglionic sympathetic nervous system 2. Primary ciliary dyskinesia (PCD) is an exception since at least one of the genes encodes a structural protein of the cilia. hence. It causes vasoconstriction. It causes an increase in metabolic rate. 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. in the heart. It activates the baroreceptor reflex. it is the most common form of a group of disorders called Neuronal Ceroid Lipofuscinoses (or NCLs). As pulmonary pressures fall after birth. worsening seizures. The inheritance 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. May cause a reflex bradycardia when given by infusion 4. Is produced by the adrenal cortex 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 there is a biochemical defect. although coronary perfusion may be increased.B. 171-Noradrenaline 1. myocardial oxygen consumption will also be increased. Decreased prostaglandin levels 3.PEDIATRICS EXAMINATION REVIEW 5. It acts predominantly via the beta adrenoceptor Noradrenaline is a catecholamine produced by the adrenal medulla. Functional closure of the foramen ovale in the first 24 hours 2. leading to a rise in blood pressure. Its actions are mediated mostly through the alpha-adrenoceptor. It also acts as a neurotransmitter in the post-ganglionic sympathetic neurons and parts of the brain. Decrease in pulmonary artery resistance following closure of the ductus arteriosus 4. Factors influencing closure of the ductus include:1. Causes a rise in metabolic rate 5. and progressive loss of sight and motor skills.

In selective IgA deficiency there is an increased incidence of cows' milk allergy.PEDIATRICS EXAMINATION REVIEW 2. 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. A2 & secretory forms). Spontaneous recovery is a recognised feature 5. affecting the levels of IgA (A1. Hyperactivity in childhood 4. Is usually asymptomatic 3.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 deficiency is common (1:350) but the incidence is not universal worldwide (rarer in Japan 1:1. GI and GU infections. 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. Intra uterine growth retardation 5. Alternatively. Maxillary hyperplasia 3. but occasionally there is an increased incidence of respiratory.jejunal biopsy should be performed. Is crossed by the attachment of the transverse mesocolon 4. pre. Most people are completely asymptomatic. Is supplied by branches of the coeliac artery alone 3.and post. May delay diagnosis of coeliac disease 4. 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.800). 175-Abnormalities associated with foetal alcohol syndrome include:1. Is crossed by the left renal vein 5. but also IgM. Hypotonia 111 . 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. The levels of IgG & A are predominantly affected. Is seen in Kartagener's syndrome 2. Short palpebral fissures 2. anaphylaxis. 174-IgA deficiency:1. autoimmune disease and malignancy.

5. The greater the intake of alcohol the more severe the signs in the neonate. There is some evidence to suggest that alcohol may interfere with transfer of essential amino acids and zinc. 4. irritability in neonatal period. Activated protein C resistence 3. Haemoglobin SS 5. Has bilateral cortical representation. 2. homocysteinuria 178-The following statement(s) concerning inherited immunodeficiency syndromes are true: 1. maxillary hypoplasia. Nephrotic syndrome 2. which are necessary for protein synthesis.. Moya moya disease Teaching Notes for Question 177 Theme: Cerebrovascular Disease Strokes are rare in childhood. Has a sensory supply in the ear canal. cardiac defects (mainly septal defects). Causes: -sickle cell disease -arteriovenous malformations and vascular dysplasia eg moya moya disease -vasculitides eg SLE. micrognathia. Due to arterial occlusion. PAN -coagulopathies eg AT III. When lesioned below the level of the stylomastoid foramen will cause hyperacusis. moderate to severe mental retardation. -dehydration -trauma -metabolic disorders eg MELAS. facial abnormalities such as short palpabral fissures. Receives taste fibres from the anterior 2/3rd of the tongue via the greater petrosal nerve. Systemic lupus erythematosis 4. and thin upper lip. hypotonia. 3. 176-The seventh cranial nerve: 1. 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). Receives taste fibres from the palate. microcephaly. 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. Up to 32% of infants born to heavy drinkers demonstrate congenital malformations. 177-Strokes in childhood are associated with :1. Teaching Notes for Question 176 Theme: The seventh cranial nerve The difference between the pattern of facial weakness seen in lower and upper motor neurone lesions is explained by the presence of bilateral cortical representation. The commonest inherited form of severe combined immune deficiency is x-linked 2. activated protein C resistence. hyperactivity in childhood. The incidence of lymphoid malignancy is increased in ataxia telangiectasia 112 .PEDIATRICS EXAMINATION REVIEW 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. venous thrombosis. epicanthic folds. protein C and S deficiency. Characteristic features include IUGR.

Patients have impaired cell mediated immunity. Management of an injury should include encouraging bleeding. HBV and HCV. washing and disinfecting the wound and reporting the incident. 3. Malaria. by sharps awareness training. 5. There is thus impaired T-cell differentiation as the interleukins are important T-cell growth and differentiation factors. 180-Leptospirosis:- 113 . Deficiency of either of these enzymes leads to the accumulation of toxic metabolites which severely affect lymphocyte production and function. Other clinical features include partial oculocutaneous albinism and abnormal platelet function. HIV. ADA and PNP are enzymes which catalyze the metabolism of purines via the uric acid pathway. The enzyme also functions in the respiratory burst . Clinically. Tuberculosis. Cytolytic T-cell-mediated killing is normal. Hepatitis B. Teaching Notes for Question 179 Theme: Needlestick injury Over 20 infections have been shown to be transmissible by needlestick injury. The commonest autosomally inherited forms are adenosine deaminase and purine nucleotide phosphorylase deficiency.the clinical picture resembles a mild form of chronic granulomatous disease.a process important for intracellular killing by phagocytes. characterised by giant granules in phagocytes due to a genetic defect in intracellular vesicle formation and hence impaired intracellular killing following phagocytosis. 179-The following diseases may be transmitted by needlestick injury: 1. Hepatitis C. Lymphoid malignancies as well as carcinomas occur with with increased frequency in these patients. low IgE. This pathway maintains glutathione in a reduced state protecting the red cell membrane from oxidant stress . Chediak-Higashi syndrome is an autosomal recessive disorder. use of safer needles. Deficiency in glucose-6-phosphate dehydrogenase results in impaired motility of leukocytes 5. a protein that is important in DNA repair. IgA and IgG2 and are prone to respiratory infections and skin infections. X-linked hyper IgM syndrome is due to the lack of CD40 ligand (CD40L) expression on activated T-cells. 4. Ataxia telangiectasia is due to defects in ATM. Serum IgM levels are high with deficiency of IgG and IgA. Complete G6PDH deficiency results in susceptibility to chronic bacterial and fungal infection . The patient may be approached for consent for testing for HIV. G6PDH is an important enzyme in the hexose monophosphate shunt. the commonest inherited form is X-linked.PEDIATRICS EXAMINATION REVIEW 3. but NK killing is impaired. The defect in Chediak-Higashi syndrome is in T-cell activation 4. Prevention is key. This leads to impairment in immunoglobulin isotype switching and germinal centre 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. sharps bins and so on. There is a mutation in the gene encoding the common gamma chain – a chain shared by receptors for a number of interleukin cytokines. The molecular defect in X-linked hyper IgM syndrome is in the expression of CD40 on B-cells Teaching Notes for Question 178 Theme: Inherited immunodeficiency syndromes Several defects can lead to the phenotype of severe combined immunodeficiency. Other known genetic defects that lead to severe immunodeficiency include mutations of DNA-dependent protein kinase (DNA-PK) and recombination activating genes (RAG). 2. these patients are particularly susceptible to opportunistic lung pathogens such as Pneumocystis carinii. with attendance at Occupational Health. Humoral immunodeficiency occurs because of a lack of T-cell help.

Is a valveless system 2. Most recover within two weeks without treatment 2. Is more common around the New Forest Teaching Notes for Question 180 Theme: Leptospirosis Diagnosis relys on demonstration of leptospira in blood and CSF. Na+ 3. from the interstitial fluid. 182-The following have a much lower concentration in the cerebrospinal fluid (CSF) than in the cerebral capillary blood. The thoracic ducts drain into the venous circulation at the junction of the sub-clavian veins and internal jugular veins 4.PEDIATRICS EXAMINATION REVIEW 1. 181-The lymphatic system: 1. Osmolarity 4. A minority of patients develop meningitis. Has a total lymph drainage of 6-8 litres of fluid/24 hours from interstitial fluid 5. * Valves then direct flow towards the thoracic ducts in a uni-directional fashion. cholestatic jaundice and renal failure (Weil's disease). the renal failure of Weil’s disease can recover. Protein 5. via contact sports (L hardjo). * Distension by the lymphatic fluid causes contraction of this smooth muscle. The Na+ concentration is roughly the same or slightly more in CSF and the K+ 114 . Maintains a uni-directional flow of lymph towards the thoracic ducts 3. is about 2-4 liters/24 hours. 1. May be complicated by thrombotic thrombocytopenic purpurea 5. * The total lymph drainage for an adult. The disease affects predominantly men. and if treatment is prompt.icterohaemorrhagiae). or serologically. Is treated with tetracycline 4. Treatment: penicillin. May be diagnosed on urine culture 3. or exposure to rat urine (L. 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. the organism gains entry via mucous membranes or cuts. 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. and is commonly acquired through occupational contact (L hardjo from cattle). K+ 2.

115 . Is always abnormal 2. muscular dystrophies or myopathies.g. 185-Conn's syndrome: 1.PEDIATRICS EXAMINATION REVIEW concentration is slightly less in CSF. Serum creatine kinase should be measured 4.g. 3. 2. Leads to increased levels of plasma angiotensinogen 2. Is due to atrophy of zona glomerulosa. in brain and spinal cord) due to extensor hypertonus and tightness of tendo achilles. Sucralfate and phenytoin 4. The antimuscarinic effect of tricyclics reduces gastric emptying and delays absorption. Leads to sodium retention. Metoclopromide increases gastric emptying and is used in combination with analgesics in migraine to enhance their absorption. Causes muscle weakness. 4. 183-Co-administration of the following pairs of drugs results in reduced absorption of the latter:1. 184-Toe-walking in an 18 month old child :1. 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. Metoclopramide and paracetamol Teaching Notes for Question 183 Theme: Absorption Aluminium and magnesium (in antacids) form insoluble complexes with tetracyclines. Duchenne muscular (where initially due to proximal weakness and pelvic instability shifting the centre of gravity forward and later to tightness of the tendo achilles) dystrophy. Spinal cord imaging may be indicated 3. Creatine kinase is a muscle enzyme and is increased in conditions with increased muscle turnover e. iron and prednisolone. Antacids and prednisolone 2. Is more common in females. Toe walking may be normal but this is a diagnosis of exclusion. Does not need to be investigated in a girl 5. Cholestyramine and warfarin 3.e. Amitriptyline and digoxin 5. 5. ii) upper motor neurone lesions (i. Toe walking may be a feature of diplegic cerebral palsy. The CSF sugar content is about two third than that of blood.

Erosive arthritis affecting the hip/knee/wrist and 2nd and 3rd MCP joints occurs. acute rheumatic fever and hypocomplementaemic vasculitis. leading to excess aldosterone production. Phagocytic cells have surface receptors that recognise the Fc portion of immunoglobulin as well as complement components. Menstruation protects homozygous women from the effects of iron overload. There is an associated Vitamin C deficiency. They mount a rapid response that does not require clonal expansion.B14 rather than B8.a process termed opsonisation. 187-Phagocytic Cells :1. Is associated with vitamin C deficiency 2. Have receptors that recognise bacterial cell wall constituents 2. IL6 and TNF. 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. aldosterone and renin (which play a role in stimulating aldosterone production). Hemachromatosis is associated with HLA A3. Require clonal expansion to mount an effective response 3. Treatment is with venesection and desferrioxamine (one molecule of desferrioxime binds one molecule of iron). These cells phagocytose foreign particles and microbes. Phagocytes of the innate immune system recognise common motifs on invading pathogens using cell surface pattern recognition receptors. They are also important in clearing apoptotic cells and debris in the resolution phase. Is more common in men 4. These cells initiate and amplify the adaptive immune response by producing pro-inflammatory cytokines such as IL1. 116 . 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. Binding of these receptors to their ligands enhances phagocytosis of antigen .PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 185 Theme: Conn's syndrome Conn's syndrome is common in males and is associated with hypertrophy of zona glomerulosa. Wilson's disease is associated with a locus is on chromosome 13.DR3. Are important for the resolution of inflammation 5. Is associated with HLA B8 5. as does chondrocalcinosis in 50 percent of cases. Release cytokines wich affect the adaptive immune system 4. the aldosterone level is elevated whilst the renin level is low or undetectable. In Conn's syndrome (primary hyperaldosteronism). Is associated with Jaccoud's arthropathy 3. Its other name is primary hyperaldosteronism. The best method of diagnosis is to measure the blood levels of two hormones. Jaccoud's arthropathy is nonerosive and associated with SLE. 186-Haemochromatosis:1. Internalised microbes are killed by phagocytes which secrete reactive oxygen intermediates and possess potent microbicidal properties.

Immune complexes are also found in SLE. Infective endocarditis 3. Vision is commonly decreased. the iris tissue is hypoplastic. Two thirds of the cases are dominantly inherited and 1/3rd are sporadic. Corneal and lens abnormalities may occur and glaucoma occurs in 75% of patients with aniridia. Teaching Notes for Question 188 Theme: Aniridia In aniridia. Wilm's tumour is associated with familial aniridia. Takayasu’s arteritis. serum sickness. 4. There is an association of anirida. Two thirds of the cases are sporadic. Red light absorbs more oxyhemoglobin than deoxyhemoglobin 2. Wilm's tumour may occur in 1/5th of patients with sporadic aniridia. They transmigrate by binding to PECAM and similar adhesion molecules. Goodpasture's disease 2.PEDIATRICS EXAMINATION REVIEW Phagocytic cells enter tissues by rolling on selectins and binding via integrins to ICAM (and similar molecules). The readings from pulse oximeters are affected by anemia 4. Macular and optic nerve hypoplasia are common and leads to visual impairment. It is bilateral in 98% of cases. There are no circulating immune complexes. 3. 5. It is associated with partial deletion of long arm of chromosome 11. Plasmodium malariae infection Teaching Notes for Question 189 Theme: Immune complex deposition In Goodpasture's disease. hepatitis B antigenemia. It is usually bilateral. 189-Immune Complex deposition in the kidney occurs in: 1. mental retardation and a partial deletion of the short arm of chromosome 11. Water vapor will cause inaccurate readings from a paramagnetic analyzer 117 . Polyarteritis nodosa 5. genitourinary anomalies. 2. The clark electrode uses a silver/silver chloride cathode 5. 188-With regards to aniridia:1. Systemic lupus 4. etc 190-Regarding oxygen measurement: 1. circulating antibodies bind to the antigen in the glomerular basement membrane and the alveolar membrane. not usually absent. The isobestic point will vary with hemoglobin concentration 3.

4. * Dark skin. there may be midline defect of the heart (VSD. * Nail varnish/ stains on the skin or nail will also distort the readings. tracheo-esophageal fistula. Goldenhar Teaching Notes for Question 191 Theme: Congenital heart disease. cardiac (VSD). less oxyhemoglobin than deoxyhemoglobin.limb defects.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 190 Theme: Oxygen measurement * Red light absorbs. * Pulse oximeters will not give accurate readings in states of vasoconstriction or venous congestion. thumb/radius/ulna hypoplasia associated with atrial & ventricular septal defect.Regarding myasthenia gravis in childhood: 1. * Water vapor tends to affect the readings of most gas analyzers. ears &vertebrae. 191-The following syndromes/ associations are associated with congenital heart disease. CHARG association. methemoglobinemia or low oxygen tensions. * The clark or polarographic electrode features a platinum cathode and Ag/AgCl anode in an electrolyte of KCl. Septo-optic dysplasia is a midline defect consisting of hypopituiterism and optic nerve hypoplasia with absence of the septum pellucidum. Holt-Oram syndrome is an autosomal dominant syndrome consisting of limb defects. . EMG shows decremental response to repetitive motor nerve stimulation. * The isobestic point (where absorption of the two forms is the same) depends on hemoglobin concentration.The cardinal features are anomalies of the face. 2. CHARGE =Coloboma. genitourinary abnormalities. Goldenhar Syndrome is associated with VSD.ASD &Tetralogy of fallot. The diagnostic test is subcutaneous endophonium. The neonatal form is always due to maternal antibodies. Vacterl association. 3. 4.oesophageal atresia. high carbon monoxide levels. Septo-optic dysplasia. in addition they may be renal anomalies. 192. choanal atresia). and Coarctation of the aorta. anal atresia. renal. heart defects (Tetralogy of fallot. 2. coarctation of the aorta. 5. PDA&tetralogy of fallot VACTERL=Vertebral. hence oxygenated blood looks red.and transposition of the great arteries. The most common associated heart defects are VSD. PDA. Thyroid dysfunction is a recognised association. 3. jaundice or anemia will not affect the results. 1. holt-Oram syndrome. growth/mental retardation. ear abnormalities including deafness. Less common defects are truncus arteriosus and transposition of the great arteries. conotruncal malformations. ASD). Cardiac defects include VSD & ASD’s. 118 .and ventricular septal defect. neurological & musculoskeletal anomalies.

but may also be an inherited channelopathy.. Teaching Notes for Question 194 Theme: Child development. Teaching Notes for Question 192 Theme: Myasthenia Gravis. 3. 3. The disease can be confined to ocular muscles. 193. Generalised weakness is present at onset in 40% or in 20% develops within the year. Infants show preference for sweet over salty or plain water. Up to 25% of exprem babies without cerebral palsy are left handed but the reason is unclear. Left handed children are more likely to have un intentional injuries as well as a higher likelihood of developmental disorders e.g. Opinion about what is excess short acting beta agonist use between cinicians however.PEDIATRICS EXAMINATION REVIEW 5. the disease remains confined to the occular muscles. cats & dogs are equally left or right paired. Neonatal form is commonly due to maternal antibodies. 195-Persistant. with other therapies such as leukotriene inhibitors coming later in the treatment cascade. Sense of taste is present from birth. conjugated hyperbilirubinaemia may be caused by: 1. Patient education is emphasized. 25% of children are left handed. In the new BTS/SIGN asthma guidelines LABA therapy is recommended at step 3 before increasing inhaled steroid dose. High dose regular short acting bronchodilators should be tried before inhaled steroids. 4. This is a surprise as animals e. 194.g. Some patients with mild or exertional asthma can be controlled on short acting beta agonists alone. All patients should be on inhaled steroids. 119 . Teaching Notes for Question 193 Theme: Treatment of asthma. 9 studies with serevent and fluticasone and one with eformoterol and budesonide confirm that steroid LABA combination is as effective inhaled steroid dose. 10% of juvenile cases have associated hyperthyroidism. 5. Prevalence rate of thumb-sucking in children are universal across races. 5. Left handedness is more common in exprem babies without cerebral palsy. Left handed children are more likely to have un intentional injuries. Alpha-1-antitrypsin deficiency.The following statements regrd the treatment of asthma: 1. Sense of taste is prersent from birth.Regarding child development: 1. Either long acting beta-2 agonists (LABAs) and low dose steroid or high dose inhaled steroid can be used to the same clinical effect. 2. It tends to be less where children are carried by parents. 4. Prevalence rates of thum-sucking in children vary from 45% of US children to 1% in eskimos. It is thought to be due to antecedent brain injury. 2. 7-10% of children are left handed. Leukotrene inhibitors are recommended in step 4. In 40% of childhood myasthenia.dyslexia and autism.

and inferior oblique). A complete oculomotor palsy results in the affected eye being “displaced down and out” with a dilated pupil. -Metabolic disorders –cystic fibrosis. -Miscellaneous – idiopathic neonatal hepatitis. hepatitis. UTI. Hypothyroidism. 3. 196-Complete transaction of the oculomotor nerve results in : 1. galactosaemia. rubella. 4. 197-The following involve deficiencies in T-cell immunity: 1. 2. levator palpebrae superioris and sphincter pupillae (parasympathetic) enter the orbit through the superior orbital fissure \9from kumar and clark). Bruton’s agammaglobulinaemia. chromosomal abnormalities. all cases need further investigation. Unconjugated neonatal jaundice is normally physiological and resolves spontaneously. 4. hypoadrenalism. -Congenital infections (STORCH-syphlis. Constriction of the pupil. toxoplasma. 18 and 13. Efferent fibres to four external ocular muscles (superior. The nucleus of the third nerve lies ventral to the aqueduct in the midbrain. Convergent squint. biliary atresia. Di George syndrome.septicaemia. -Endocrine disorders – hypothyroidism. inferior and medial recti. Teaching Notes for Question 196 Theme: Oculomotor nerve. 4. choledochal cyst. 3. Wiskott-Aldrich syndrome. inspissated bile syndrome. Ptosis.antitrypsin deficiency. lysosomal storage disorders. -Anatomical disorders – intrahepatic:bile duct hypoplasia. Teaching Notes for Question 195 Theme: Neonatal jaundice. Loss of accommodation and light reflex. 5. 3. 2. trisomy 21. Causes of conjugated hyperbilirubinaemia in a neonte are: 1)-Intrahepatic cholestasis -Infections.PEDIATRICS EXAMINATION REVIEW 2. Cytomegalovirus infection. cytomegalovirus. spontaneous bile duct perforation. herpes virus infection) -Acquired infections. Diplopia. 120 . hypopituitarism. 2)-Extrahepatic cholestasis. Cystic fibrosis. peroxisomal disorders. Chronic granulomatous disease. 5. alpha-1. fructosaemia. Haemolytic disease.

Usually before the age of 2. Wiskott-Aldrich syndrome is associated with humoral and T-cell immunodeficiency. Toxoplasmosis. Crdinal features are thrombocytopenia. Sleepwalking: Occur in a state of deep. 3. Night terrors: child wakes up. Alternative of the parents responses to the children’s night time behaviour so as to prevent reinforcement of the problem. Occur in up to 3% of toddlers. The underlying defect in Di George syndrome is failure of normal development of the 3rd and 4th pharyngeal pouches leading to aplasia or hypoplasia of the thymus and associated disorders. Breath Holding spells: Common in preschool children. Sleep disorders are never treated with drugs. Chronic mucocutaneous candidiasis. 199-The following maternal conditions are known to cause adverse effects on the neonate: 1. Di George syndrome and chronic mucocotaneous candidiasis are predominantly Tcell defects. 2. there is a minor epileptic seizure. Diabetes insipidus. Drug treatments not thought to be particularly effective. Night terrors occur in the stages of deep sleep. frightened and inaccessible. Chronic granulomatous disease is due to defective neutrophil killing. Microvilli on T-cell appear abnormal. Breath holding attacks should be investigated with CT scan. eczema and immunodeficiency. Precipitated by minor upsets. not responding when spoken to. 4. Period of disturbed behaviour may last up to 15 minutes. Chronic mucocutaneous candidiasis presents with persistant candidal infection of the nails. Occur on waking from deep sleep . Reinforced when parents show concern. 5. 198-In young children. The defect is a deficiency in the wiskott-aldrich protein – which play a role in cytoskeletal organization and possibly intracellular signaling. The child then starts to breath in half a minute or so. Btk is necessary for transducing signals initiated by ligation of the pre-Breceptor. the child goes blue in the face especially around the face.PEDIATRICS EXAMINATION REVIEW 5. 3. Chronic myeloid leukaemia. Child eventually goes back to sleep. unlike nightmares that occur in REM or light sleep. Parental reassurance. May last for a few minutes to half an hour. 2. skin and mucus membranes. Bruton’s agammaglobulinaemia is an X-linked recessive B-cell (humoral) defect due to mutation in the btk gene. T-cell numbers and function are normal. T-cell numbers are reduced. Breath holding attacks cause cyanosis. Teaching Notes for Question 198 Theme: Sleep disorders. Teaching Notes for Question 197 Theme: Deficiencies in T-cell immunity. Follow crying which increases in intensity until breathing stops. may be difficult to comfort. 1. This gene encodes a protein tyrosine kinase – B-cell tyrosine kinase (Btk). usually in expiration. Occasionally. Can be used by children to express anger towards parents. 121 . All Ig levels are low and mature B-cells and plasma cells are present. Behavioural treatments best. Sleep walking occurs during REM sleep.

3-DPG concentrations in rbcs. and the associated decrease in affinity of haemoglobin for oxygen contributes to the transfer of oxygen from the blood in to the tissues. Alkalosis will have the opposite effect. High altitude. The greater affinity of 122 . 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. 2.Temperature – A rise in temperature shifts the curve to the right i. Haemoglbin is composed of four globin subunits (in normal adults – two alpha and ywo beta globin chains) each of which contains a haem group. Increased 2-3 DPG.High altitude results in a rise in 2. 3. 2. The rate of transmission is +/. 5. A rise in 2. the neonate may present with thyrotoxicosis due to the transplacental passage of TSI.3-DPG shifts the oxygen dissociation curve to the right releasing more O2.e as long as the circulating antibodies persist in the baby’s circulation.2. Alkalosis. The skin is frequently involved with malar rashes and there can be haematological and cardiac abnormalities. 3. 4. Increased red cell volume and MCH. Teaching Notes for Question 200 Theme: Oxygen uptake. Maternal chronic myeloid leukaemia can have adverse effect on pregnancy – fetal/neonatal mortality is 16-38%. Toxoplasmosis leads to congenital infection by transplacental transmission. 5.PH – a fall in PH shifts the curve to the right i. The ewborn presents with the classic triad of hydrocephalus. 200-en uptake by haemoglobin is characterized by: 1. with an increase in the availability of oxygen to the tissues. If hyperthyroidism is due to Grave’s disease or Hashimoto thyroiditis.PEDIATRICS EXAMINATION REVIEW 4. 4. SLE. The most frequent heart abnormality is congenital heart block – 90% of mothers whose infants have congenital heart block are antiRo(SSA)Ab positive. Teaching Notes for Question 199 Theme: Maternal conditions causing adverse effects on the neonate. anaemia and infectious complications. The neonate presents with clinical features of SLE due to transplacental passage of maternal Ab. This curve is affected by a number of factors: 1. This is known as the Bohr effect. The oxygenhaemoglobin dissociation curve relates the saturation of haem with oxygen as a % (the y axis) to the PO2 in mmHg(on the x axis).this substance is formed by glycolosis via the EmbdenMeyerhof pathway.3-DPG levels.60% of third trimester infections and 20%-30% during the first two trimesters. chorioretinitis. Haem is a complex of prophyrin and ferrous iron.e oxygen uptake by haemoglobin is decreased. and cerebral calcification. It binds to the beta-globin chains of deoxyhaemoglobin and causes oxygen to dissociate from Hb.e a higher PO2 is required for haemoglobin to bind oxygen. Raised temperature. Splenomegaly can restrict intrauterine growth and lead to premature delivery. These symptoms are frequentely short-lived i. 99% of the oxygen which dissolves in blood is transported by the oxygen carrying compound haemoglobin. Pathology can be secondary to placental leukaemic infiltrates. Hyperthyroidism.

In anaemia. Questions (PART 2) SELECT ONLY ONE ANSWER 123 . and the curve is right shifted. the MCHC is reduced. rather than the MCH or MCV. 5.With regards rbc indices. increasing the release of oxygen to the tissues. it is the MCHC which affects the oxygendissociation curve.PEDIATRICS EXAMINATION REVIEW foetal haemoglobin for oxygen as compared to its adult counterpart is a result of the decreased binding of 2.3-DPG by the foetal gamma globin chain.

Hyperbaric oxygen B. Anti-smooth muscle antibodies in Primary biliary cirrhosis C. Anti liver cytosol antibodies in primary sclerosing cholangitis 4-hich one of the following cells in the lung parenchyma produces surfactant? Options: A. He is confused. Anti-mitochondrial antibodies in type II autoimmune hepatitis E. vomiting.A 14-year-old boy is rescued from a burning building and is brought to the hospital. 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. Type I pneumocyte E. Endothelial cell C. Goblet cell D. Urgent senior surgical opinion to exclude bowel obstruction 2-Low protein C levels are not seen with: Options: A. High flow oxygen via a facial mask C. Which of the following is the best treatment for this patient? Options: A. Anti-liver/kidney microsomal antibodies (anti LKM-1) in type I autoimmune hepatitis B. Chicken pox E. Factor V Leiden D. He has nausea. CT brain and lumbar puncture D.PEDIATRICS EXAMINATION REVIEW 1. Warfarin therapy B. Anti-nuclear antibodies in Primary biliary cirrhosis D. Alveolar macrophage B. Type II pneumocyte 124 . diarrhoea and abdominal pain. The carboxyhaemoglobin (COHb) level is 25%. Neonatal purpura fulminans C.

Yellow fever is endemic in India and South-East Asia. Interferon-1-Beta C. C. Azathioprine D. Grade IV lupus nephritis B. Lung transplantation B. Visceral leishmaniasis is not found in the southern European countries. B. High -dose steroids 125 . Cyclophophamide E. Malaria is only endemic in the tropics. 7-The best treatment option for a patient with Usual Interstitial Pneumonia (UIP or CFA (Cryptogenic fibrosisng alveolitis) is :Options: A. Post-streptococcal glomerulonephritis C. IgA nephropathy (Buerger’s disease) 6-Which of the following statements about the epidemiology of tropical diseases is correct? Options: A. E. Subacute bacterial endocarditis D.PEDIATRICS EXAMINATION REVIEW 5-Which of the following conditions with renal involvement is the most likely to have normal complement levels:Options: A. D. Type II cryoglobulinaemia E. The distribution of infection with Schistosoma mansoni and Schistosoma haematobium are similar. Chaga's disease is confined to South America.

Haemolysis following transfusion of blood bearing mismatched blood group antigens C. Wiskott Aldrich syndrome 10-All of the following are type II hypersensitivities except:Options: A.PEDIATRICS EXAMINATION REVIEW 8-What is the diagnosis that can you infer from the X-ray abdomen? Options: A. He is below the 2nd centile for weight. SBO (Small bowel obstruction) C. Adenosine deaminase deficiency D. Toxic Megacolon D. He has had 3 previous admissions to hospital with pneumonia and has required two courses of antibiotics for otitis media. LBO (Large bowel obstruction) B. What is the most likely underlying diagnosis :Options: A. The Arthus reaction D. C4 deficiency E. Ataxia telangiectasia C. Production of autoantibodies to acetylcholine receptors in myasthenia gravis B. Hyperacute graft rejection E. Primary antibody deficiency B. Chagas Disease E. Quinidine-induced thrombocytopenia 126 . Ankylosing Spondylitis 9-A 6 month old boy with severe eczema presents with easy bruising and two nose bleeds.

It is a cystic disease of lung. Kown to exist in 4 genetic forms 12-Which of the following statements regarding toxoplasmosis in pregnancy is false? Options A.PEDIATRICS EXAMINATION REVIEW 11-Polycystic kidney disease is: Options A. Characterised histologically by micro. Risk of fetal infection is increased with increasing gestational age. It is associated with pulmonary hypoplasia C. Foetal abnormality may occur in cases of maternal infection occurring 6 months pre-conception.and macroscopic cysts distributed throughout the renal parenchyma E. Subdural haematoma B. Sturge-Weber syndrome C. D. It usually presents with vomiting 15-Regarding Histiocytosis X :Options: A. Usually fatal at term B. bone and anterior pituitary 127 . C. The radiologist reports the presence of intracranial calcification. Characterised histologically by tubule dysplasia and cystic tissue throughout the renal parenchyma C. 13-A 9-year-old has a cranial CT scan. Ataxia telangiectasia E. It is most commonly seen on the right side B. Which of the following diagnoses is least likely to be associated with the radiologist’s report? Options: A. E. Toxoplasmosis D. It is always symptomatic at birth E. It is associated with oligohydramnios D. B. Hypoparathyroidism 14-Choose the one correct statement regarding a Congenital diaphragmatic hernia:Options A. Maternal toxoplasma-specific IgM may persist for over a year. Treatment with pyrimethamine and sulphadiazine is recommended during the first trimester to reduce risk of foetal infection. Documented seroconversion confirms infection. Always clinically apparent at birth D.

C. most are men 20-40 years old D.PEDIATRICS EXAMINATION REVIEW B. Ageing is thought to be due to shortening of the telomeres. The data are positively skewed B. Ageing can be reduced in mammals by caloric restriction. Down's Syndrome D. William's syndrome C. E. 80% of patients are smokers. Which is the best conclusion to draw from the data? Options: A. The mean is the best measure of central tendency D. It is a rare cause of pneumothorax 16-A girl with an unusual facial appearance is found to have coarctation. Ageing is caused by nitric oxide free radical-induced mitochondrial damage. 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. Possible diagnoses are: Options: A. Ageing can be slowed in animal models by free radical scavengers. Most important treatment strategy is steroids E. Noonan's syndrome B. Ageing is associated with altered insulin signalling. B. 128 . Edward's syndrome 17-A study measures the heights of 13-yr-old children. CXR shows small lungs with prominent markings and honeycombing C. The median is greater than the mode E. Turner's syndrome E. The data are normally distributed C. D.

Shunt nephritis D. Left lingular collapse E. 21-Which of the following renal disorders is associated with normal serum complement levels? Options: A. 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. 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. Left lingular consolidation D. A proto-oncogene is transformed onto an oncogene by changing the protein-coding segment or by altering its expression. Membranoproliferative glomerulonephritis B. Right lower lobe consolidation 20-Which one of the following is true regarding cancer genetics?:Options: A. RB. E. Post-infectious glomerulonephritis E. Left lower lobe consolidation B.PEDIATRICS EXAMINATION REVIEW 19-What is the diagnosis? Options: A. and BRCA1 are oncogenes C. Goodpasture’s disease 129 . All forms of lupus nephritis C. B. Left lower lobe collapse C. A gene whose product is involved either in transforming cells in culture or in inducing cancer in animals is called a proto-oncogene. D. APC.

Once initiated. the half-life of which is several days C.2 mmol/l HCO3 36 mmol/l Ca 2.4 mmol/l P04 1. it follows a stereotypical cascade. Ectopic atrial tachycardia 130 . Atrial flutter B. a potent chemokine B. Causes the release of C8a. Anorexia nervosa E.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. AV reentrant tachycardia due to accessory pathway E. Ehlers Danlos syndrome 23-A patient presents with acute infective arthritis. Causes the release of CRP. The resulting inflammation: Options: A. Supraventricular tachycardia C. Is always harmful E. Persistent vomiting D.0 mmol/l Urine chloride 10 mmol/l Options: A. Sinus tachycardia D. Primary hyperparathyroidism B. Primary hyperaldosteronism C. which is not regulated 24-What does this ECG of an 8-year-old boy with Ebstein’s anomaly show? Options: A. May be defined as increased vascular permeability accompanied by an infiltration of inflammatory cells D.

Neuromuscular symptoms are similar to those in hypocalcaemia D. Axillary freckling B. Lesch-Nyhan syndrome B. the following statement is not true: Options: A. Café au lait patches 28-Choose the one correct statement regarding necrotising enterocolitis: Options: A. Lisch nodules E. It is not seen in babies who haven't been started on feeds yet D. Hungry bone syndrome is associated with reduced serum magnesium 27-All these are major clinical features of neurofibromatosis-1 (NF-1) except: Options A. It usually involves the lower colon B.Choose the correct statement regarding alpha 1-antitrypsin (AAT): Options: 131 . Severe exfoliative psoriasis E. Thiazide diuretic therapy C. It may lead to intestinal obstruction a few weeks later E. Prolonged vomiting is an indication for high-dose oral magnesium supplementation E. It can be prevented by the use of oral antibiotics 29. Tibial pseudoarthrosis C. High-dose aspirin therapy 26-In a patient with hypomagnesaemia. Recent cisplatin treatment could have been the cause B.PEDIATRICS EXAMINATION REVIEW 25-Which of the following conditions is not associated with hyperuricaemia :Options: A. Hypocalcaemia may occur C. Polycythaemia rubra vera D. Cortical tubers D. It is commonly associated with gas in the biliary tree C.

The low levels of the enzyme in deficiency syndromes are due to lack of synthesis of the variant enzyme E. TFTs normal What is the underlying diagnosis ? Options: A. PO4 0. It is associated with cataplexy B. It may be associated with hypnopompic hallucinations 132 . It is associated with early-onset REM sleep E. She has recently started developing breasts. Cystic fibrosis D.9 U&E normal. AST 34 Ca2+ 1. The affected pupil is always larger than the unaffected B. Over 75% of cases of emphysema in the UK are associated with the deficiency of this enzyme C.5 . Global areflexia is a recognised association E. Coeliac disease C. Both pupils may be affected C. Hb 10. It exhibits X-linked inheritance pattern C. ALP 320 MCV 108 Bili 12.7 . Ptosis is rare 31-A 17 year old girl presents with primary amenorrhoea. Addison’s disease E. Both her sisters (age 14 and 19) are greater then 170cm tall and are well.6 . It is a membrane bound tyrosine kinase B. She is 145 cm tall and weighs 48 kg. Night time insomnia can be a feature D. WCC 7. Crohn’s disease B. Deficiency presents as cirrhosis in children D. 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.PEDIATRICS EXAMINATION REVIEW A. There is denervation supersensitivity of the pupil D. Ulcerative colitis 32-Which of the following is not true of narcolepsy? Options: A.

Pneumococcal pneumonia D. The technique can be used to map the position of genes on a chromosome 35-Cardiac tamponade may occur with all of the following except: Options: A. Tuberculosis B.PEDIATRICS EXAMINATION REVIEW 33-The following are all true about migraine in children except: Options: A. Rheumatic fever C. EEG will help in the diagnosis C. Production of immunoglobulin with higher affinity to the antigen C. More than 50% of headaches are unilateral B. An aura and postdrome stage is present as in adult patients E. Isotype switching to IgG B. The technique is used instead of light microscopy for routine chromosome analysis E. Requirement of help from T-cells 133 . The technique requires specialised microscopes C. 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. Uraemia 36-The secondary immunoglobulin response is not characterized by:Options: A. A quantitatively increased antibody production as compared to the primary immune response E. Production of memory plasma cells D. The technique can detect small deletions in DNA D. Rheumatoid arthritis E. The technique can be performed on uncultured cells B. Other members of the family may be affected D.

C. with the doctor called when the blood sugar reaches 4mmol/l. neck and hands. Serum C-peptide will not be raised in exogenous insulin use E. c-reactive protein and erythrocyte sedimentation rate C. A 72hr fast should be organised. Urine sulphonylurea levels are unhelpful B. Anti nuclear antibody and c-reactive protein B. Complement levels 134 . What are the most appropriate tests to request? Options: A. joint aches and a red rash on her face.PEDIATRICS EXAMINATION REVIEW 37-What is the abnormality seen in this girl with bone pain? Options: A. Subperiosteal resorption of HPTH (Hyperparathyroidism) B. HPOA (Hypertrophic Osteoarthropathy) C. glucose and C-peptide should be taken every morning for 7 days D. A family history of scars in the neck is irrelevant 39-A 16-year-old girl presents with fever. Myelomatous deposit E. Full blood count and chemistry D. Serum insulin. She attributed the rash to severe sunburn and says that she has sensitive skin. Osteopetrosis 38-In the investigation of a patient who is admitted following 3 dizzy spells each markedly improved by eating:Options: A. Anti-nuclear antibody and erythrocyte sedimentation rate E. AVN (Avascular necrosis) talus D.

PEDIATRICS EXAMINATION REVIEW 40-Which of the following is true about prolactin? Options A. Iridocyclitis usually responds to topical steroids. D. He also has bilateral sensorineural deafness with a sister and younger brother who are similarly afflicted. Steroids are the agents of first choice. Levels are increased by cimetidine 41-Choose the incorrect statement. Gold salts may lead to nephrotic syndrome. Audiometry E. B. Elevated levels produce gynaecomastia without galactorrhoea B. Damage to the pituitary stalk reduces prolactin levels C. C. What is the most useful investigation? Options: A. E. Hydroxychloroquine is associated with ocular toxicity. Anti-GBM antibodies D. Can stand alone D. Urine microscopy B. Serum creatinine C. Renal biopsy 43-The following statement is false regarding treatment of Juvenile Idiopathic Arthritis (JIA):Options A. Can crawl C. Low dose methotrexate may be beneficial 135 . By the age of 9 months a child: Options A. Levels are elevated in >90% of cases of acromegaly E. Can sit with support B. 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. Metoclopramide increases prolactin production D. Can play peek-a-boo E.

Consolidation right lower lobe B. Collapse lingular lobe D.PEDIATRICS EXAMINATION REVIEW 44-What is the abnormality shown on Chest X-ray? Options: A. Collapse right lower lobe C. Perforation E. Bilateral pneumothoraces 136 .

C1q deficiency and angio-oedema C. A raised C4 and low C3 in SLE is associated with active disease 137 . C5-C9 deficiency and mycoplasma infection B. Proliferative DM retinopathy B. Is more common in women than in men. Is associated with haemolytic crises induced by sulfonamides. Endocarditis D. Background retinopathy 46-All of the following statements about Glucose-6-phosphate dehydrogenase deficiency are false except: Options: A. Often leads to chronic haemolysis. Requires treatment with desferrioxamine to prevent iron overload. C2 deficiency and rheumatoid arthritis D. CMV retinitis E. B. D. C3 def is associated with partial lipodystrophy E. Grade 3 hypertensive retinopathy C. C.PEDIATRICS EXAMINATION REVIEW 45-What is the diagnosis? Options: A. Is caused by mutations of the G6PD gene on chromosome 4. E. 47-Which of the following statements correctly describes the clinical phenotypes which are associated with a deficiency of the various complement components? Options: A.

Anti-mitochondrial antibodies in Primary biliary cirrhosis D. capillary refill time is 5 seconds and he has a purpuric rash over his lower limbs. Anti-phospholipid antibodies in Primary biliary cirrhosis C. Secure intraosseus access and give 20mL/kg fluid D. Check blood sugar C. Continue to try for IV access and then give 20mL/kg fluid if successful B. A colleague has been trying for 5 minutes to secure intravenous access when you arrive. Give immediate IM ceftriaxone before securing IV access E. His heart rate is 160/min. Fourth heart sound B. Third heart sound C. ANCA in type II autoimmune hepatitis E. On examination. Systolic thrill E. He has been unwell for 12 hours with fever and has vomited once. Where is the lesion likely to be? Options A. Facial nerve D.PEDIATRICS EXAMINATION REVIEW 48-The following autoantibodies are commonly found in the named disease: Options A. There is also a ptosis present in this eye. Cervical sympathetic trunk 138 . Anti-liver cytosol antibodies in primary sclerosing cholangitis 49-All of the following are clinical features of severe aortic stenosis except: Options: A. The next step should be: Options: A. He is not responsive to pain. Trigeminal nerve C. Check a capillary blood gas 51-A patient is noted to have a small left pupil. Length of murmur in systole 50-A two-year-old boy is brought to the resuscitation room. Soft aortic component of second heart sound D. Anti-liver/kidney microsomal antibodies (anti LKM-1) in type I autoimmune hepatitis B. Vagus nerve E. His mother has noted that the left side of his face is dryer than normal. Oculomotor nerve B. his airway is patent and he is breathing oxygen via a mask with non-rebreathing bag with oxygen saturations of 100%.

Which of the following is NOT a symptom of Horner’s syndrome? Options: A. Those with chronic renal failure D. A larger sample should have been used B. Those with chronic heart disease C. Can be terminated with intravenous adenosine E. Is usually left atrial in origin B. Usually causes a narrow complex tachycardia at 150 bpm C. The trial would have been better run against placebo 55-Which of the following is correct regarding atrial flutter? Options: A. Those with diabetes mellitus E. Immunosupressed people due to disease or treatment B. What is the best conclusion to draw? Options A. Implies the presence an accessory pathway 139 . The p-value associated with this statin means that it is a clinically significant new drug E. The alternative hypothesis should be accepted C. Absence of sweating on face and neck E. Critical level is set at 0. Pupillary constriction B.01 and difference is found (p = 0. 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.PEDIATRICS EXAMINATION REVIEW 52-A patient is diagnosed as having a Horner's syndrome following carotid surgery. Is often associated with an irregular pulse D.001). The probability of a type I error occuring is 1% D. Those with coeliac disease 54-The lipid-lowering effect of a new statin is compared to atorvastatin. Ptosis C.

The chest X-ray shows oligaemic lung fields C. Left pneumothorax B. Left lingula collapse D. TOF is associated with dextroposition of the aorta E. Left lower lobe collapse C. if no contraindications. Left hilar adenopathy 140 . Children with TOF are susceptible to cerebral abscess B. E. A renal consultation is advised if the serum creatinine rises above 250 µmol/l.PEDIATRICS EXAMINATION REVIEW 56-The following statements are true about Tetralogy of Fallot (TOF) except: Options: A. The second heart sound is soft and single D. B. This disorder results in a left to right shunt 57-In terms of the NICE guideline of screening for diabetic nephropathy in Type 2 patients: Options: A. D. All patients with confirmed microalbuminuria. Calculating ratios of albumin and creatinine in the urine is not as useful as the total urine protein level. C. The target HbA1c is about 8%. Left tension pneumothorax E. Blood pressure is less important than reducing the degree of proteinuria. 58-What can you infer from the Chest X-ray? Options: A. should be on an ACE inhibitor.

5–10% of cases manifest with meconium ileus at birth B. Staphylococcus aureus and pseudomonas aeruginosa are two pathogens most commonly isolated from the sputum of patients with this condition 141 . a patient’s axillary nerve is damaged. the genetic abnormality is a four-base pair deletion at the locus on chromosome 7 C. C. His mother reveals that when he was 2 years old he was diagnosed as having ‘malabsorption’. a 10-year-old boy presents with a chronic cough. and she has noticed that his sweat tastes excessively salty. A loss of sensation may occur in the lateral forearm.PEDIATRICS EXAMINATION REVIEW 59-Which of the organs below is considered an immunologically privileged site :Options: A. B. Pancreas 60-Following a dislocated shoulder in a rugby match that gets out of hand. recurrent lung infections and clinical evidence of obstructive pulmonary disease. What is the likely result? Options: A. Thymus C. The teres major atrophies. The rounded contour of the shoulder disappears. Testicle E. 61-Steve. Bone marrow D. The primary defect is in the transport of chloride ions across epithelial tissue E. Pancreatic abnormalities are present in 80% of patients D. All the above are features of this condition EXCEPT: Options: A. The patient may have a wrist drop. Skin B. D. The patient may lose the ability to adduct the arm. E. In 70% of patients.

Pulmonary embolism D.8 mmol Clotting screen: PTT APTT VDRL 11 seconds 39 seconds Positive 1:320 Drug screen of urine Plasma alcohol Negative Not detected What is the likely diagnosis? Options: A. Her only medication is the OCP. Syphilis E. The C5 spinal root. D.9 g/dl MCV 83 fl WCC 3. blood 2+ proteins. arthralgia and headache and a red. 64-A boy presents with fever. He recently visited his GP for a deep IM steroid injection.1 mmol/l Creatinine 178 mmol/l Albumin 30 g/l Total protein 80 g/l LFTs Normal BM 7.PEDIATRICS EXAMINATION REVIEW 62-A 26-year-old airhostess is brought to the A&E department following a witnessed grand mal seizure. * There is slight puffiness of her fingers and she has low-grade pyrexia of 37. C. E. SLE B. * Fundoscopy reveals silver wiring and AV nipping bilaterally. Systemic sclerosis 63-An elderly man presents to casualty with a wrist drop and pain radiating down his right arm. 142 . She has been previously fit and well except for a history of mild intermittent arthralgia affecting both hands. Her blood test results reveal: Hb 9.1 mmol/l Urea 10. The medial cord of the brachial plexus. Epilepsy C. The long thoracic nerve. annular rash in his axilla. The probable site of the injection was the: Options: A.6C. The ulnar nerve. * On examination she has no focal neurology. * BP 160/98 mmHg.1 x 109/l Platelets 117 x 109/l CRP 2 g/l ESR 71 mm in the first hour Sodium 134 mmol/l Potassium 5. She smokes 20 cigarettes per day and drinks 15 units of alcohol per week. Posterior cord of the brachial plexus. B. * Urinalysis tests 3+.

Which is the most likely diagnosis? Options: A. He should receive the acellular pertussis vaccine when next immunised D. The presence of jaundice would make which one of the following diagnoses more likely? Options: A. Kawasaki disease B. Toxocariasis D. He should receive the oral pertussis vaccine when next immunised E. Scarlet fever D. Lyme disease E. On examination. Lyme disease E. Sydenham’s chorea C. Huntington’s disease D. What will you recommend for this child in the future? Options A. Infectious mononucleosis C. Hemiballismus B. He should receive the whole cell pertussis vaccine (as DTP) as usual at his next immunisation 68-You see a 15-year-old girl in clinic with the following results: * Hepatitis B surface antigen: positive 143 . Torticollis E. Malaria C. Rheumatic fever 65-Milkmaid grasp is characteristically seen in which of the following conditions? Options A. most of the anterolateral surface of the thigh is red and indurated. Gilles de la Tourette syndrome 66-A 2-year old boy is admitted with fever. He should not have any further immunisations B. He should have all immunisations except pertussis C.PEDIATRICS EXAMINATION REVIEW which his mother says has been present for one week and has increased in size. 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. Typhoid B.

Plasma urate levels are elevated C. Plasma sodium is usually normal B. B. Heart failure. Urine osmolality is >500 mosm/kg E. Splenic rupture. Serum globulins are high 70-A 14 year-old boy is seen in the Ambulance and Emergency Department. 72-Acute attacks of porphyria:- 144 . E. Liver failure. Kidney failure. Na 135.29 . Previously vaccinated against hepatitis B 69-In the syndrome of inappropriate ADH secretion (SIADH): Options: A.5. The following blood test results are found. Acute hepatitis B infection B.PEDIATRICS * * * * EXAMINATION REVIEW 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. Pco2 4. MDMA 71-The most important cause of death in infectious mononucleosis is: Options: A. Ethylene glycol C. Chronic hepatitis B infection (high infectivity) E. Cl 110. Past hepatitis B infection C. pH 7. He is unconscious and he has a rash around his mouth. Opiates D. Chronic hepatitis B infection (low infectivity) D. Oedema may occur due to fluid overload D. D. Methanol B. Glue E. C. Respiratory failure. HCO3 15. What has he taken? Options: A. K 5.

Posterior cord of the brachial plexus B. Are a feature of porphyria cutanea tarda D. Anterior uveitis C. Optic neuritis B. he reports having only central vision over an area of approximately 1 m by 1 m. 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. Can produce hyponatraemia due to Diabetes Insipidus C. “Non-organic” symptoms E. The probable site of the injury was the:Options: A.PEDIATRICS EXAMINATION REVIEW Options: A. Methanol poisoning D. Are precipitated by the contraceptive pill E. Which of the following diagnoses best fits his symptoms? Options: A. On examination. T1 spinal root D. right arm and shoulder during a game of tennis. Ulnar nerve 145 . He reports this at a distance of 3 m and also at 6 m. C5 spinal root E. Can be precipitated by paracetamol B. Are associated with malabsorption 73-A 17-yr-old male medical student presents with a sudden onset of tunnel vision. Medial cord of the brachial plexus C.

but at an OPD appointment 3 months later is complaining of worsening right hip pain poorly responsive to NSAIDs. O2 sats 94% on room air.8 mmol 11 seconds 39 seconds positive 1:320 negative not detected The patient then develops acute dyspnoea and examination reveals decreased air entry at the right base.1 X 109/l 117 X 109/l 2 g/l 71 mm in the first hour 134 mmol/l 5. 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. Fundoscopy reveals silver wiring and AV nipping bilaterally.1 mmol/l 10. BP 160/98 mmHg.PEDIATRICS EXAMINATION REVIEW 75-A 26-year-old air hostess is brought to A&E following a witnessed grand mal seizure. Urine analysis tests show 3+ blood. BP 105/55. Her 24-hour urinary free cortisol is 450 nmol. Measuring cortisol levels D. she smokes 20/day and drinks 15 units of alcohol per week. High-dose dexamethasone suppression test C. Septic arthritis D.6°C. Trochanteric bursitis 76-A 35-year-old woman who complained of easy bruising. There is slight puffiness of the fingers and low-grade pyrexia of 37. She has been previously fit and well except for a history of mild intermittent arthralgia affecting both hands. Measuring ACTH levels E. Avascular necrosis C. Low-dose dexamethasone suppression test B. What is the most likely cause of her hip pain? Options: A. tachycardia. Osteoporosis B. CRH stimulation test 146 . Stress fracture E. X-ray is normal but bone scan shows increased uptake in the hip. On examination she has no focal neurology. She responds well to prednisolone and azathioprine. Which is the next investigation to confirm the diagnosis? Options: A.9 g/dl 83 fl 3. Her only medication is the OCP. weight gain and striae is being worked up for Cushing’s syndrome.1 mmol/l 178 mmol/l 30 g/l 80 g/l normal 7. 2+ protein.

Rifampicin D. a 16-year-old boy develops fever and 147 . He has been vomiting non-billious breast milk for the last 3 weeks. Strict barrier nursing is essential to contain the infectious agent B. Pneumocystis carinii D. CXR shows hazy shadowing in the right mid and lower zones. She is given treatment with prednisolone and cyclosporin. Therapy is started and 5 days later he becomes acutely jaundiced with red discolouration of the urine. On examination the child is afebrile and dehydrated with a depressed anterior fontanelle. Ciprofloxacin E. Cryptococcus neoformans 78-A 6 week old male baby presents with a 2 day history of multiple seizures. iv 5% dextrose D. iv isolyte – P C. Tetracycline 81-Seven weeks after returning from rural Ghana. iv Hypertonic saline 79-Regarding Legionnaire's disease:Options: A. The intravenous (iv) fluid of choice for administration would be: Options: A. Cytomegalovirus E. Klebsiella pneumoniae C. iv 50% calcium gluconate B. Examination reveals no focal signs of infection. Cefotaxime B. Which of the following drugs is the likely cause :Options: A. however after 48 hours the fevers continue and a repeat CXR shows left lower lobe consolidation. Pontiac fever is a rarer and milder form of the disease E. Preexisting immunocompromise is a risk factor C. Ultrasound of the abdomen shows hypertrophic pyloric stenosis. iv sodium bicarbonate E. Streptococcus pneumoniae B. Options: A. Diagnosis can be made from stool culture 80-A 15 year old boy is admitted with pneumonia. headaches and a non-productive cough. Penicillin C. however 10 days after admission she develops a fever. Blood cultures are taken and a CXR is unremarkable.PEDIATRICS EXAMINATION REVIEW 77-A 26-year-old woman is admitted to hospital for cadaveric renal transplantation. diarrhoea. She is treated with cefotaxime. Investigations show hyponatraemia and mildly deranged LFTs. Rifampicin alone is as effective as erythromycin for the treatment D. The operation is uneventful however she is admitted to the Intensive Therapy unit for 48 hours postoperatively.

Phenytoin. Cushing's syndrome. The pupil appears dilated and does not constrict to light. Both parents should have karyotype analysis. Blood cultures E. Moxonidine. CMV retinitis B. Congenital adrenal hyperplasia. B. IgM for viral haemorrhagic fever C. visual acuity on the left is down to light perception. Amaurosis Fugax E.PEDIATRICS EXAMINATION REVIEW rigors. She complains of discomfort in the eye and thinks that difficulty with perception of colour was the first problem that she noticed. Which of the following statements is most accurate? Options: A. On examination. Amniocentesis should only be offered to confirm the diagnosis of Down’s syndrome if the family will consider termination of the pregnancy D. Chest radiograph D. Most children with Down's syndrome are born to women over the age of 35 years C. but there is no need to karyotye the affected child B. Cyclosporin A. E. A karyotype of 46XX excludes a diagnosis of Down’s syndrome E. although does when a torch is shone in the right eye. Thick and thin blood film B. Central retinal artery occlusion 148 . Anterior iscaemic optic neuropathy D. D. Which of the following would not be implicated in the aetiology? Options: A. They have one child affected with Down’s syndrome and are considering another pregnancy. C. What is the most likely diagnosis :Options: A. The least useful test is: Options: A. 83-A family attends genetic counselling. during a trip to an art gallery. Optic neuritis C. 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 days. HIV antibody test 82-A 15-year-old is undergoing investigation for hirsuitism.

8g/dl plats 210 X 109/l WCC 4.9 X 109/l plasma glucose 6. Familial Mediterranean fever B. and rash. Prednisolone B. Behcet’s syndrome D. Pyridostigmine D. photophobia and neck stiffness. Familial Hibernian fever E.1 mmol/l no organisms seen on microscopy 48 hour culture of CSF negative What is the diagnosis? Options: A. He is lucid with a GCS of 15. Non Hodgkin’s lymphoma 86-Which of the following do not tend to make the symptoms of myasthenia gravis worse? Options: A. ANA. pleuritic pain. He also describes recurrent episodes of fever.PEDIATRICS EXAMINATION REVIEW 85-A 29 year old Cypriot man gives a history of recurrent pain and swelling of the left knee. His blood test results show: Hb 13. Aminoglycosides C.5 mmol/l CRP 39g/l ESR 56 mm in the first hour RF. ENA and ANCA negative CSF analysis: opening pressure 14cmH2O protein 0.6 g/l RBC 2 /ml WBC 21/ml (100% lymphocytes) CSF glucose 4. Lithium E. He is then hospitalised with severe headache. Adult onset Still's disease C. Quinidine 149 .

Mitral stenosis 88-A patient has hypothyroidism. Pulmonary thromboembolic disease B. Fallot’s tetralogy C. Eisenmenger’s syndrome E. which muscle would you look for wasting? Options: A. In view of the probable aetiology. Is inherited as an autosomial dominant trait D. Flexor digiti minimi E. Abductor pollicis brevis C. Serum cerruloplasmin levels are raised B. She develops a painful hand at night. Transposition of great arteries D.PEDIATRICS EXAMINATION REVIEW 87-This 15-year-old was cyanosed on exertion. Medial two lumbricals D. May result in retinal abnormalities E. which of the following statements is true :Options: A. Most common presentation is with a manic depressive illness 150 . What is the diagnosis? Options: A. Lateral two interossei B. Extensor pollicis 89-In a patient that has been previously diagnosed with Wilson’s disease. When penicillamine is administrated then urinary copper level rise C.

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. Hypervolaemia is a common problem C. and 1+protein. He has more than 46 chromosomes B. It is a notifiable disease C.PEDIATRICS EXAMINATION REVIEW 90-Which of the following statement(s) regarding childhood minimal-change nephrotic syndrome is/are true?:Options. and having soaking wet napping. There is an increased risk of him having a child with difficulties 92-In leptospirosis which one of the following is true? Options: A. 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. Nephrocalcinosis.3}. D. drinking 3-4 bottles of water through the night. urine dipstick shows 1+ glucose. The patient may be jaundiced during the immune phase D. 151 . Cataract. Albumin is routinely given to children with proteinuria D. Options: A. t (2. On immunofluorescent studies. Which of the following features are you likkely to find on examination and investigation.5)(q35. Which of the following statements is correct?:Options: A. 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. Long-term cyclophosphamide therapy is beneficial in the steroid resistant group E. It is usually transmitted by rat bite B. Corneal opacities. Metabolic acidosis with a wide anion gap. A. E. The organism may be isolated from urine during the septic phase E. deposition is seen along the glomerular basement membrane B. Her parents tell you that she has always been a very thirsty child.p21. C. Hyperkalaemia. He is likely to be infertile E. B.

Thrombocytopaenia. Skin necrosis. B. D. Sickle cell diseas(HbSS). 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. Peripheral neuropathy. This is positive deposite a normal Hb & MCV. C. D.Which of the following is not a recognised complication of anticoagulation therapy? Options: A. Deep venous thrombosis. Start IV fluids & allopurinol. B. Commonce chemotherapy straight away. Do a lymph node biopsy to exclude concomitant lymphoma.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. Referre him for a bone marrow transplantation. B. The Hb-electrophoresis shows HbA 55% and Hbs 45%. Sickle-B-thalassaemia. but there are small blasts in the blood film. his full blood count is normal . 95. Haematologically normal. B-thalassaemia trait. Osteoporosis. E. and Hb-electrophoresis shows an HbA band only with HbA2 quantitation of 4. C. Discharge him and review in 1 week time as an out patient. C. E. D. E. a bone marrow aspirate confirm that he has acute lymphoblastic leukaemia. 152 . Her partner has a microcytic anaemia.8%. Sickle cell trait.

presence of postural hypotension. 153 .4 urea=10. Ig A nephropathy. 98-A 18 years old student becomes un well with a sore throat. C. C.50). If a child has tried many treatments over a number of years the prognosis is poor. there is a 1% chance that he will still bed-wet. C.31g/l (0.Stomach distension increases colonic motility. What is the most likely diagnosis? Options: A. Mucous.20)C4= 0. investigations reveals: FBC normal . D.1 . on examination he has flacid weakness predominantly in the legs and absent reflexes. protein +. B.CH50= 87% (50-110) Urinalysis: blood 3+. Runs in families. Henoch-Schonlein purpura. E. which one of the following statement(s) regarding enuresis is not true? Options: A. Evidence demyelination on nerve conduction studies. low grade fever and has some cervical lymphadenopathy. B. E. At 15 years age. Increased parasympathetic activity leads to decreased colonic motility. Na and Cl are actively secreted.20-0. E. on consulting his GP 3 days after the onset of this illness. C. B. CSF examination shows markedly raised protein & a mild lymphocytosis. K and bicarbonate are absorbed.PEDIATRICS EXAMINATION REVIEW 97-A 9 years old male has always wet the bed. examination is otherwise unremarkable.creatinin =141 . There is a 30% chance that his father was a bed-wetter. History of diarrhea in the preceding few weeks. D. It is the site of most vitamin C absorption. E. 99-An adolescent male is admitted with progressive limb weakness. Sever weakness. few red cell casts. he has no daytime enuresis. Interstitial nephrosis. Which of the following does not imply a worse prognosis? Options: A.Na=137 K=4.551. Drinking tea causes bed-wetting D. B.42g/l (0. post-streptococcal glomerulonephritis.C3= 1. Wegener’s granulomatosis. Markedly reduced FVC. 100-Only one of the following is correct regarding physiology of the colon: Options: A. D.

PEDIATRICS EXAMINATION REVIEW Answer &Teaching Notes for Questions (PART 2) 1.A 14-year-old boy is rescued from a burning building and is brought to the hospital. He 154 .

Intravenous fluid and oxygen via nasal prongs E. cardiac complications.(protein C levels <1% of normal) present with severe neonatal purpura fulminans. 2) Causes of acquired protein C deficiency: * infections . The carboxyhaemoglobin (COHb) level is 25%. * heterozygous deficiency (protein C levels 30-40% of normal) most frequently presents after puberty with deep venous thrombosis of the lower limb. and consequently a thrombophillic state. Factor V Leiden D. which becomes activated by thrombin/thrombomodulin complexes on the surface of endothelial cells. Hyperbaric oxygen B. Neonatal purpura fulminans C. Most thrombotic episodes occur spontaneously but known associated risk factors include surgery with immobilisation.g. A positive family history of thrombosis is associated with an increased risk of symptoms . 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. vomiting. They can present with massive venous thrombosis as older children.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.acute myeloid leukaemia * chronic inflammation . Which of the following is the best treatment for this patient? Options: A. cerebral thrombosis and DIC. will experience a thrombotic event. COHb levels of >40% and pregnant women 2-Low protein C levels are not seen with: Options: A.+ . High flow oxygen via a facial mask C. It requires protein S as a cofactor. 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. 5-fluorouracil). chemotherapeutic agents e. 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. pregnancy and the oral contraceptive.PEDIATRICS EXAMINATION REVIEW has nausea. Activated protein C inhibits coagulation by degrading coagulation factors Va and VIIIa and resulting in decreased thrombin formation. It circulates in an inactive form. Many people remain asymptomatic. Those with milder defects have protein C levels of 10 -24 % of normal.meningococcal disease and varicella infection * DIC * hepatic disease * drugs . The indications for hyperbaric oxygen are neurological or psychiatric symptoms. Warfarin therapy B. CT brain and lumbar puncture D. Chicken pox E. Causes of protein C deficiency are: 1) Congenital * homozygous deficiency . It can also present with recurrent superficial thrombophlebitis and DVT. cyclophosphamide. methotrexate.50% of individuals with heterozygous protein C deficiency and a family history of thrombosis. diarrhoea and abdominal pain. He is confused. Protein C levels are not decreased 155 . lasparaginase * malignancy .warfarin.those with severe deficiency .

5-Which of the following conditions with renal involvement is the most likely to have normal complement levels:Options: A.classical complement pathway and immune complex formation 156 .Anti liver/kidney microsomal antibodies (LKM-1) Anti liver cytosol Primary Biliary Cirrhosis . Anti smooth muscle antibodies.Anti nuclear antibodies. Type I pneumocytes. Grade IV lupus nephritis B. Its key function is gaseous exchange.PEDIATRICS EXAMINATION REVIEW 3-Choose the correct matching pair of disease and antibody out of the following options:Options: A. 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. Type II cryoglobulinaemia E. Anti-mitochondrial antibodies in type II autoimmune hepatitis E. Anti liver cytosol antibodies in primary sclerosing cholangitis C Teaching Notes for Question 3 Theme: Antibodies in GI diseases Autoimmune hepatitis type I . Alveolar macrophage B. Anti mitochondrial (M2) antibodies Primary Sclerosing cholangitis . The other conditions are all associated with activation of the . Anti-liver/kidney microsomal antibodies (anti LKM-1) in type I autoimmune hepatitis B. Subacute bacterial endocarditis D. Endothelial cell C. 4-hich one of the following cells in the lung parenchyma produces surfactant? Options: A. Anti-smooth muscle antibodies in Primary biliary cirrhosis C. Post-streptococcal glomerulonephritis C. Anti mitochondrial antibodies type II . Type II pneumocyte E Teaching Notes for Question 4 Theme: Surfactant production Type II pneumocytes produces surfactant in the lungs. which are as many as type II pneumocytes. Goblet cell D.ANCA positive . Anti-nuclear antibodies in Primary biliary cirrhosis D.Anti nuclear antibodies. cover 95% of the epithelial surface area. Type I pneumocyte E.

Italy and Greece Schistosoma haematobium and S.Yellow fever occurs in Africa and South America only Malaria is endemic in large areas of the south shore of the Mediterranean.Northern India and South East Asia. E. Interferon-1-Beta C.PEDIATRICS EXAMINATION REVIEW 6-Which of the following statements about the epidemiology of tropical diseases is correct? Options: A. 157 . Chaga's disease is confined to South America.mansoni are both endemic in Africa. Teaching Notes for Question 6 Theme: Epidemiology of tropical diseases . Visceral leishmaniasis is not found in the southern European countries. Lung transplantation B. The distribution of infection with Schistosoma mansoni and Schistosoma haematobium are similar.latter is also found in South America 7-The best treatment option for a patient with Usual Interstitial Pneumonia (UIP or CFA (Cryptogenic fibrosisng alveolitis) is :Options: A. 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. Cyclophophamide E. which lie north of the Tropic of Cancer .Chagas' disease is found mainly in South America . Malaria is only endemic in the tropics. D. B. but the . . Single lung transplantation for UIP results in an actuarial survival of 80% at one year and 55% at three years. Azathioprine D.Visceral leishmaniasis is endemic in parts of Spain. Yellow fever is endemic in India and South-East Asia.

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. C4 deficiency E. Chagas Disease E. What is the most likely underlying diagnosis :Options: A.cell interaction and platelet function.PEDIATRICS EXAMINATION REVIEW 8-What is the diagnosis that can you infer from the X-ray abdomen? Options: A. 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).and T. Adenosine deaminase deficiency D. He has had 3 previous admissions to hospital with pneumonia and has required two courses of antibiotics for otitis media. This is a cytoskeletal associated protein which is required for B. LBO (Large bowel obstruction) B. Primary antibody deficiency B. and phagocytes of WAS patients show defects in the 158 . platelets. He is below the 2nd centile for weight. Ataxia telangiectasia C. Toxic Megacolon D. In vitro studies with T-cells. Ankylosing Spondylitis Teaching Notes for Question 8 Theme: Toxic megacolon Large bowel thick walled and dilated. SBO (Small bowel obstruction) C.

They can also bind to cell surface receptors interfering with normal function. thrombocytopaenia and recurrent bacterial infections. These children can present with lifethreatening bleeding. Hyperacute rejection is usually due to preformed anti-donor antibody. 10-All of the following are type II hypersensitivities except:Options: A. normal levels of IgG and high levels of IgA and IgE. There is impaired cell mediated immunity (with lymphopenia) and a characteristic pattern of immunoglobulin levels:. malignant disease. There is also reduced expression of CD43 (sialophorin) on the surface of Band T-lymphocytes. Kown to exist in 4 genetic forms 159 . Patients present with progressive neurological deterioration. Type II hypersensitivity reactions involve antibody-mediated destruction of cells. Myasthenia gravis. Characterised histologically by tubule dysplasia and cystic tissue throughout the renal parenchyma C. autoantibodies bind to acetylcholine receptors at the neuromuscular junction. Host antibodies (usually IgM) react with foreign antigens on the incompatible transfused blood cells and lead to complement-mediated haemolysis of these cells. Patients have recurrent respiratory infections and are susceptible to organisms such as Pneumocystis carinii and herpes simplex virus. These antibodies are directed against foreign MHC molecules of other alloantigens 11-Polycystic kidney disease is: Options A. Characterised histologically by micro. This interferes with normal motor end plate function and also results in a reduction in receptor numbers. Ataxia telangiectasia is caused by defective DNA repair mechanisms. Antibody usually IgG or IgM can destroy cells by activating complement. and the major defect is impaired production of antibody to thymus independent polysaccharide antigen.structures which are affected by actin reorganisation. Patients present with eczema. ataxia. or promoting phagocytosis of antibody-coated cells. Hyperacute graft rejection E. The Arthus reaction D. In this disease.and macroscopic cysts distributed throughout the renal parenchyma E. Later lymphocyte numbers decrease. The Arthus reaction is a type III hypersensitivity reaction mediated by the local deposition of immune complexes with consequent complement activation and neutrophil infiltration. drug-induced thrombocytopenia and hyperacute graft rejection are all examples of type II hypersensitivity reactions. Haemolysis following transfusion of blood bearing mismatched blood group antigens C.low IgM. Initially lymphocyte numbers are normal. Bone marrow transplant is curative. and immunodeficiency becomes more severe. Platelet numbers are decreased and platelets are smaller than usual. Production of autoantibodies to acetylcholine receptors in myasthenia gravis B. autoimmune haemolysis. Always clinically apparent at birth D. Quinidine-induced thrombocytopenia C Teaching Notes for Question 10 Theme: Type II hypersensitivities. impaired cell mediated immunity and defective antibody production. Adenosine deaminase defiency causes about 20% of cases of SCID. They also have an increased risk of lymphoid malignancy. Usually fatal at term B.PEDIATRICS EXAMINATION REVIEW formation of microvilli and phagocytic vacuoles. An example of antibody interfering with normal receptor function occurs in myasthenia gravis. Blood transfusion reactions exemplify type II hypersensitivity.

Foetal abnormality may occur in cases of maternal infection occurring 6 months pre-conception. 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 (toxoplasma. Which of the following diagnoses is least likely to be associated with the radiologist’s report? Options: A. Features of congenital infection include choroidoretinopathy. Sturge-Weber syndrome C. D.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 11 Theme: Polycystic kidney disease * This condition is generally classified as autosomal dominant or recessive. Toxoplasmosis D. CMV. Teaching Notes for Question 12 Theme: Toxoplasmosis in pregnancy. The radiologist reports the presence of intracranial calcification. Infection in the mother is usually asymptomatic. today in the absence of severe pulmonary hypoplasia affected infants can be expected to survive beyond the neonatal period. the consequences of infection are greater during early pregnancy. * Neither type is always obvious at birth. Although foetal infection risk is greater during late gestation. microcephaly and intracerebral calcification with psychomotor retardation. Pyrimethamine is contraindicated during the first trimester. astrocytoma) (viii) Vascular lesions Ataxia telangiectasia is not generally associated with intracranial calcification 160 . B. The neonate may present acutely with petechiae. 13-A 9-year-old has a cranial CT scan. Subdural haematoma B. Maternal toxoplasma-specific IgM may persist for over a year. Ataxia telangiectasia E. chronically IgM positive with low levels lasting up to 2 years post-infection. * Histologically it is characterised by the presence of cysts. and nor is either form universally fatal. E. 12-Which of the following statements regarding toxoplasmosis in pregnancy is false? Options A. Documented seroconversion confirms infection. maculopapular rash and hepatomegaly. the recessive form not being apparent often until adulthood. C. Infected women may be ‘serofast’. Treatment with pyrimethamine and sulphadiazine is recommended during the first trimester to reduce risk of foetal infection.and hyperparathyroidism (vii) Tumours (craniopharyngioma. but not renal dysplasia. TB. Risk of fetal infection is increased with increasing gestational age. abscesses) (iv) Tuberose sclerosis (v) Sturge-Weber syndrome (vi) Hypo.

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EXAMINATION REVIEW

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

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Teaching Notes for Question 16
Theme: Unusual facial appearance and aortic coarctation
Turner’s syndrome is associated with aortic coarctation.
Noonan’s syndrome has a phenotype similar to Turner’s, but is AD, thus meaning equal sex
distribution. In addition, Noonan’s syndrome is associated with pulmonary stenosis and
HOCM, not coarctation.
William’s syndrome has associated supravalvular aortic stenosis.
Facial features of Turner’s syndrome include: webbed neck, high-arched palate and low
posterior hairline.

17-A study measures the heights of 13-yr-old children.
Which is the best conclusion to draw from the data?
Options:
A. The data are positively skewed
B. The data are normally distributed
C. The mean is the best measure of central tendency
D. The median is greater than the mode
E. 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. In general, it is always good to remember that:
1. Mode is towards the hump
2. Mean is towards the tail
3. Median is in the middle
Best way of summarising skewed data is to give the median and interquartile range. A
Normal distribution is best summarised by quoting the mean (for its middle point) and
standard deviation (for its spread). A skewed distribution is best summarised by quoting
the median (for its middle point) and interquartile range (for its spread).
18-Which of the following hypotheses on the mechanisms of ageing is least accurate?
Options:
A. Ageing is caused by nitric oxide free radical-induced mitochondrial damage.
B. Ageing is associated with altered insulin signalling.
C. Ageing can be slowed in animal models by free radical scavengers.
D. Ageing can be reduced in mammals by caloric restriction.
E. Ageing is thought to be due to shortening of the telomeres.

Teaching Notes for Question 18
Theme: Mechanisms of ageing
Ageing is complex and the mechanism unclear. It is thought to occur due to superoxideinduced damage to structures such as mitochondria, particularly by reactive oxygen
species. This has been disputed by the finding that mitochondria from the elderly brain are
functional.

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In animal models, a slowing of ageing can be induced by caloric restriction even in
mammals and by free radical scavenging. Studies in invertebrates have identified genes
that alter lifespan and which also encode components of insulin signalling pathways.
Examples include DAF-2 (caenorhabditis elegans) and InR (Drosophila).
A recent study using knockout mice implicates the mammalian insulin-like growth factor
receptor as an important regulator of lifespan. Another prominent hypothesis for ageing is
that associated with telomere shortening.

19-What is the diagnosis?
Options:
A. Left lower lobe consolidation
B. Left lower lobe collapse
C. Left lingular consolidation
D. Left lingular collapse
E. Right lower lobe consolidation

Teaching Notes for Question 19
Theme: Lower lobe consolidation
* No loss of volume and there is airspace shadowing, therefore, it is consolidation.
* Left paraspinal line (and left hemi-diaphragm) obscured, therefore, left lower lobe.

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20-Which one of the following is true regarding cancer genetics?:Options:
A. 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.
B. RB, APC, and BRCA1 are oncogenes
C. A gene whose product is involved either in transforming cells in culture or in inducing cancer in
animals is called a proto-oncogene.
D. 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.
E. A proto-oncogene is transformed onto an oncogene by changing the protein-coding segment or by
altering its expression.

Teaching Notes for Question 20
Theme: Cancer genetics
No teaching note available.
21-Which of the following renal disorders is associated with normal serum complement
levels?
Options:
A. Membranoproliferative glomerulonephritis
B. All forms of lupus nephritis
C. Shunt nephritis
D. Post-infectious glomerulonephritis
E. Goodpasture’s disease

Teaching Notes for Question 21
Theme: Complement levels and glomerular disease.
Causes of low C3 levels include:
1. Post-infectious glomerulonephritis
2. Lupus nephritis
3. MPGN
4. Shunt nephritis
5. Infective endocarditis with renal involvement
6. Type II cryoglobulinemia
Both infective endocarditis and shunt nephritis are immune complex mediated disorders
with activation of the complement pathway and resulting hypocomplementemia.
Note that Focal segmental glomerulosclerosis is not associated with low C3 levels.
Similarly, rapidly progressive GN is a clinical diagnosis and is not necessarily
hypocomplementemic.
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 3.2 mmol/l HCO3 36 mmol/l Ca
2.4 mmol/l P04 1.0 mmol/l Urine chloride 10 mmol/l
Options:
A. Primary hyperparathyroidism
B. Primary hyperaldosteronism
C. Persistent vomiting
D. Anorexia nervosa
E. Ehlers Danlos syndrome

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Causes the release of C8a. Sinus tachycardia D. It acts as an opsoninenhancing phagocytosis. The resulting inflammation: Options: A. It is secreted in increased amounts within 6 hours of an acute inflammatory stimulus. C-reactive protein binds to phosphocholine moieties found on microbial polysaccharides. Supraventricular tachycardia C. a potent chemokine B. The resolution of inflammation is tightly regulated. rubor and dolor of the inflammatory process. which is not regulated Teaching Notes for Question 23 Theme: Acute inflammation. CRP has a half-life of about 12 hours. 24-What does this ECG of an 8-year-old boy with Ebstein’s anomaly show? Options: A. Ectopic atrial tachycardia 165 . AV reentrant tachycardia due to accessory pathway E. Atrial flutter B. The combination of increased vascular permeability and cellular influx gives rise to the calor. Is always harmful E. May be defined as increased vascular permeability accompanied by an infiltration of inflammatory cells D. 23-A patient presents with acute infective arthritis. and also activates the classical complement pathway. Once initiated. it follows a stereotypical cascade. indicating volume depletion. the half-life of which is several days C. C5a is a complement intermediate which acts as a potent chemokine. Acute infection results in activation of complement. Causes the release of CRP. CRP is an acute phase protein which is synthesised by hepatocytes as a result of the acute inflammatory process.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 95 Theme: Acid-base disorders She has a metabolic alkalosis with hypokalaemia and a low urine chloride.

Combined mechanisms: -Alcohol consumption . Causes are: -Renal insufficiency -Acidosis .g. whereas high-dose aspirin can have a therapeutic effect in gout. -Glycogenoses types III.enhanced renal tubular reabsorption of uric acid. Endogenous uric acid comprises roughly two thirds of total body urate. decreased tubular secretion. dissociated atrial and ventricular activity is never seen as both atria and ventricles are part of the same circuit.results in accelerated hepatic breakdown of ATP and generation of organic acids that compete with urate for tubular secretion. and VII can result in hyperuricaemia due to excessive degradation of skeletal muscle ATP. 25-Which of the following conditions is not associated with hyperuricaemia :Options: A. Approximately 70% of the urate produced daily is excreted by the kidney. while the rest is eliminated by the intestines. -Low-dose aspirin . These are negative in the inferior leads and have a rate of 300/min. -Increased purine nucleotide breakdown . starvation ketosis. cytotoxic therapy -Enzymatic defects . * In other SVTs (including those utilising accessory pathways). The remaining one third is derived from the diet. blast crisis of leukaemias. IV. -Diabetes insipidus . Polycythaemia rubra vera D. 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.due to rapid cell proliferation e. Accumulation of organic acids competes with uric acid for renal tubular secretion. The deficiency of HGPRT accelerates purine biosynthesis with a resultant increase in uric acid production.enhanced renal tubular reabsorption of uric acid. spasticity and occasionally self-mutilation.inhibition of uric acid secretion. Causes are: -Increased intake in the diet. * The next most likely diagnosis with this ECG is ectopic atrial tachycardia.an inherited X-linked disorder due to a deficiency of HGPRT . In addition to arthropathy and obstructive nephropathy.Lesch-Nyhan syndrome . these patients develop a neurological disorder that is characterised by choreoathetosis.diabetic ketoacidosis.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 24 Theme: Atrial flutter * This ECG shows classical saw-toothed atrial waves typical of atrial flutter. ethanol. or increased renal tubular reabsorption. However the typical P wave morphology and rate is pathognomonic of atrial flutter. Thiazide diuretic therapy C. 166 . Lesch-Nyhan syndrome B. The ventricular rate is typically 150/min because of 2:1 AV conduction. increased cell death rhabdomyolysis.an enzyme that catalyses the conversion of hypoxanthine to inosinic acid. -Diuretic therapy . severe psoriasis. Low-dose aspirin is associated with hyperuricaemia. High-dose aspirin therapy Teaching Notes for Question 25 Theme: Hyperuricaemia Uric acid is the final product of purine metabolism. Overproduction of uric acid: This accounts for only a minority of patients presenting with hyperuricaemia. Severe exfoliative psoriasis E.

Neuromuscular symptoms are similar to those in hypocalcaemia D. Prolonged vomiting is an indication for high-dose oral magnesium supplementation E. Axillary freckling B.neurofibromas.café au lait patches. Lisch nodules E. . 167 . Hypomagnesaemia results in defective PTH secretion and secondary hypercalcaemia. . The main features of this condition include: . .axillary freckling. .skeletal manifestations and . It usually involves the lower colon B.Lisch nodules in the eye. Tibial pseudoarthrosis C. 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. It may lead to intestinal obstruction a few weeks later E. Hypocalcaemia may occur C.a family history of a first-degree relative diagnosed with NF-1. Diagnosis of this condition is mainly done on a clinical basis with gene testing offered to a small minority.000 individuals. 27-All these are major clinical features of neurofibromatosis-1 (NF-1) except: Options A. 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. Recent cisplatin treatment could have been the cause B.PEDIATRICS EXAMINATION REVIEW 26-In a patient with hypomagnesaemia. It is not seen in babies who haven't been started on feeds yet D. * 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. It is commonly associated with gas in the biliary tree C. Cortical tubers D. Hungry bone syndrome is associated with reduced serum magnesium Teaching Notes for Question 26 Theme: Hypomagnesaemia.

this is not due to a lack of synthesis – rather a blockage of its processing and secretion from the liver. People with both PiS genes (PiSS) are less severely affected. Heterozygous states (PiMZ. Some neonates with the PiZZ genotype may develop rapidly progressive liver damage . Approximately 5% of patients die of their liver disease. Alpha 1-antitrypsin deficiency can produce jaundice and liver problems in babies within days of their birth. The consistent occurrence of overt liver disease in newborn infants. 168 . 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.an enzyme released by leucocytes during phagocytosis. Inclusion bodies in hepatocytes are a feature of AAT deficiency. it may also occur in babies who have never been fed. The majority of patients with clinical liver disease are homozygotes with a PiZZ phenotype. and brain-specific neuroserpin. emphysema results from the uncontrolled action of proteases on pulmonary tissue. with alpha 1-antitrypsin levels 60–70 per cent of normal. AAT deficiency accounts for less than 1% of cases of COPD in the UK. Although plasma levels of the enzyme are low. PiMS. Another allele (Z) results in an increased risk of emphysema. and approximately 10–15% of adult patients will develop cirrhosis usually over the age of 50 years. The normal allele (M) is seen in over 95% of Europeans. Hepatic pathology is also associated with tissue damage secondary to the accumulation of polymerised AAT. Alpha 1-antitrypsin is encoded by the protease inhibitor (Pi) locus. alpha 2-antiplasmin. is not frequently seen. The use of oral antibiotics does not reduce the risk of developing NEC. Deficiency presents as cirrhosis in children D. including luminal substrate. Cigarette smoking increases the number of neutrophils in the alveolus and inactivates the remaining small amounts of antiprotease. rapid introduction of feeds and the use of formula feeds. Other members of the serpin family include C1-esterase inhibitor. and patients with the ZZ phenotype have levels of AAT less than 20% of normal and develop severe disease. A number of factors have been implicated in the aetiology of NEC. Over 75% of cases of emphysema in the UK are associated with the deficiency of this enzyme C. Smoking is the greatest risk factor for the development of emphysema in adults with alpha 1-antitrypsin deficiency.Choose the correct statement regarding alpha 1-antitrypsin (AAT): Options: A. It is a membrane bound tyrosine kinase B. It usually involves the terminal ileum and ascending colon. Over 100 different variants of alpha 1-antitrypsin have been identified. A few children with alpha 1-antitrypsin deficiency develop significant liver disease before the age of 20. Initially.PEDIATRICS EXAMINATION REVIEW It is predominantly seen in preterms. 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. this may never develop or occur later in life. The low levels of the enzyme in deficiency syndromes are due to lack of synthesis of the variant enzyme E. hepatic pathology is often limited to minor portal fibrosis but frequently progresses to overt liver damage in late adulthood. 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. in contrast to its only occasional occurrence in young adults. However. bacterial overgrowth. 29.a clinical indication for liver transplantation. In alpha 1-antitrypsin deficiency. In non-smokers. and the protease alleles are codominant. Gas in the biliary tree although a pathognomonic sign of NEC. can be explained by the fact that the liver cells in the infants are less capable of degrading the polymerised protein.

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.9 U&E normal. There will be a compensatory rise in her parathyroid hormone levels and there is a risk of secondary hyperparathyroidism. 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. the other pupil may also become affected 31-A 17 year old girl presents with primary amenorrhoea. Coeliac disease C. Addison’s disease E. 32-Which of the following is not true of narcolepsy? Options: A. AST 34 Ca2+ 1. It is caused by idiopathic degeneration of parasympathetic neurones in the ciliary ganglion and therefore exhibits denervation supersensitivity: the affected pupil will constrict on exposure to low concentration of pilocarpine (0. Hb 10.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. It is associated with cataplexy B. Night time insomnia can be a feature D.5 . She is 145 cm tall and weighs 48 kg. PO4 0. Both her sisters (age 14 and 19) are greater then 170cm tall and are well. Global areflexia is a recognised association E.125%) whereas the unaffected will not. Crohn’s disease B. she also has macrocytic anaemia. It is associated with early-onset REM sleep 169 . She has recently started developing breasts. With time the affected pupil may become small. There is denervation supersensitivity of the pupil D.7 .6 . again suggestive of the diagnosis. Cystic fibrosis D. WCC 7. TFTs normal What is the underlying diagnosis ? Options: A. Both pupils may be affected C. ALP 320 MCV 108 Bili 12. It exhibits X-linked inheritance pattern C.

which occurs once per week or per month and is usually associated with a strong family history. The technique can be performed on uncultured cells B. 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. In many cases headache is associated with photophobia. More than 50% of cases are not unilateral. The acute headache. More than 50% of headaches are unilateral B. which presents over a short period of time with neurological deficit. An aura and postdrome stage is present as in adult patients E. blurred vision and even pain in the throat are warning signs. 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. A history of stomach pain. Other members of the family may be affected D. Sleep studies show abnormal periods of early-onset REM sleep. The headache may last from one hour to 2–3 days.PEDIATRICS EXAMINATION REVIEW E. Triptans are not licensed in children but various studies have proven their efficacy in adults. Chronic. 33-The following are all true about migraine in children except: Options: A. blurred vision and vomiting. Special microscopes using UV filters are used to detect the fluorescent signals. vivid hallucinations on falling asleep (hypnogogic) or waking (hypnopompic). 170 . The technique requires specialised microscopes C. Sleepiness responds to stimulants and cataplexy to clomipramine. It may be associated with hypnopompic hallucinations 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). non-progressive headache needs non-urgent investigation and initial treatment with simple analgesia can be commenced. and sleep paralysis. Very small rearrangements can be identified by this technique. Migraine is an acute headache. EEG will help in the diagnosis C. Sometimes a multi-disciplinary team approach is required. There is a breed of Doberman pinschers in whom the condition is dominantly inherited (AD). and usually the classical picture of migraine attack does not accompany the attack. requires urgent investigation. The technique is used instead of light microscopy for routine chromosome analysis E. 34-The following statements about techniques using FISH (fluorescent in situ hybridisation) are true except: Options: A. the affected gene encodes a protein produced in the lateral hypothalamus called hypocretin. The technique can detect small deletions in DNA D. Sleep in a dark quiet room with analgesia is often useful.

but are short lived cells in order to limit antibody responses. After priming during the primary immune response. 36-The secondary immunoglobulin response is not characterized by:Options: A. Certain bacterial antigens can activate B cells directly. 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. Therefore the secondary immune response to a specific antigen occurs more rapidly than the primary response. Memory B-cells differ qualitatively as well as quantitatively from naïve B cells. Uraemia Teaching Notes for Question 35 Theme: Cardiac tamponade All the above cause pericarditis. Memory B-lymphocytes have undergone class switch and produce mainly IgG but also other immunoglobulin isotypes. Rheumatic fever C.g. (NB. One of the main research uses of FISH is to localise genes on chromosomes. Plasma cells are the effector cells of both primary and secondary immune responses.PEDIATRICS EXAMINATION REVIEW Interphase FISH on uncultured cells is currently used in rapid prenatal diagnosis. Memory B-cells also have higher affinity for antigen as they have undergone affinity maturation in the germinal centres of lymphoid tissue. but rheumatic fever does not lead to tamponade that needs decompression. Both primary and secondary immunoglobulin responses require T-cell help. 171 . They secrete large amounts of antibody. 35-Cardiac tamponade may occur with all of the following except: Options: A. LPS). Production of immunoglobulin with higher affinity to the antigen C. Isotype switching to IgG B. and larger amounts of antibody are produced. Production of memory plasma cells D. FISH analysis is used as a second line of investigation after conventional light microscopy. the frequency of antigen-specific B-cells may increase 10-100 fold. A quantitatively increased antibody production as compared to the primary immune response E. Rheumatoid arthritis E. Pneumococcal pneumonia D. e. Tuberculosis B.

AVN (Avascular necrosis) talus D. A 72hr fast should be organised. 172 . Serum C-peptide will not be raised in exogenous insulin use E. pituitary. with the doctor called when the blood sugar reaches 4mmol/l. In multiple endocrine neoplasia type 1. A family history of scars in the neck is irrelevant Teaching Notes for Question 38 Theme: Hypoglycaemia Investigation of hypoglycaemia requires a 72hr fast. lymphoma. dysentery. bronchiectasis. Serum insulin.periosteal reaction along distal long bones. HPOA (Hypertrophic Osteoarthropathy) C. ideally. C. coeliac. metastasis * Pleural: fibroma or mesothelioma * CVS: Cyanotic congen heart disease * GIT: IBD. Causes: * Chest: CA bronchus. lymphoma. glucose and C-peptide should be taken every morning for 7 days D.PEDIATRICS EXAMINATION REVIEW 37-What is the abnormality seen in this girl with bone pain? Options: A. levels of glucose. Whipples. Urine sulphonylurea levels are unhelpful B. C-peptide and insulin are taken when BM is LOW <4. abscess. cirrhosis esp PBC Subperiosteal resorption – HPTH. Serum insulin and urine sulphonylureas levels help check for use of these drugs. 38-In the investigation of a patient who is admitted following 3 dizzy spells each markedly improved by eating:Options: A. Osteopetrosis Teaching Notes for Question 37 Theme: Hypertrophic Osteoarthropathy HPOA . Myelomatous deposit E. Subperiosteal resorption of HPTH (Hyperparathyroidism) B.

In cases of sulphonylurea-induced hypoglycaemia. plasma insulin and C-peptide are high. plasma insulin and C-peptide are high. but not galactorrhoea or elevated prolactin. Prolactin secretion is inhibited by dopaminergic stimulation carried in the pituitary stalk. Full blood count and chemistry D. and urine sulphonylurea levels are undetectable. Cimetidine has a weak anti-androgen effect. She attributed the rash to severe sunburn and says that she has sensitive skin. and sulphonylurea can be detected in urine. increased TRH (as TSH decreased) and co-secretion of prolactin mean that 30% have prolactin elevation. which increases prolactin levels. 40-Which of the following is true about prolactin? Options A. which produces gynaecomastia. joint aches and a red rash on her face. exogenous insulin administration. Metoclopramide is a dopamine antagonist. In insulinoma. c-reactive protein and erythrocyte sedimentation rate C. Damage to this increases prolactin levels. In acromegaly. neck and hands. Check ANA. Metoclopramide increases prolactin production D. and urine sulphonylurea levels are undetectable. Levels are increased by cimetidine Teaching Notes for Question 40 Theme: Prolactin Elevated prolactin levels produce gynaecomastia with galactorrhoea. Anti-nuclear antibody and erythrocyte sedimentation rate E. Also check ESR and complement levels. Causes of hypoglycaemia include insulinoma. and sulphonylurea intake. any rash in a photosensitive distribution should have SLE excluded. Elevated levels produce gynaecomastia without galactorrhoea B. Complement levels Teaching Notes for Question 39 Theme: Systemic lupus erythematosus This patient has Systemic Lupus Erythematosus. C-peptide is not elevated.PEDIATRICS EXAMINATION REVIEW pancreas. In cases of exogenous insulin administration. plasma insulin is very high. Levels are elevated in >90% of cases of acromegaly E. 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. Anti nuclear antibody and c-reactive protein B. antiDNA antibodies. 39-A 16-year-old girl presents with fever. What are the most appropriate tests to request? Options: A. injury to the stalk. 173 . Damage to the pituitary stalk reduces prolactin levels C. and parathyroid tumours occur.

Important for discussion about inheritance. Most children begin to stand alone at 12 months. then proteinuria and progressive renal impairment. Can play peek-a-boo E. Anti-GBM antibodies D. 43-The following statement is false regarding treatment of Juvenile Idiopathic Arthritis (JIA):Options 174 . Can stand alone D. Can sit with support B. By the age of 9 months a child: Options A. anterior lenticonus. 42-An 18-year-old man is referred to the outpatient clinic as his general practitioner has found him to have microscopic haematuria. Can crawl C. Diagnosis is by renal biopsy or occasionally skin biopsy. Serum creatinine C. 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). corneal lesions and rarely leiomyomata. What is the most useful investigation? Options: A. Urine microscopy B. deafness. 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. He also has bilateral sensorineural deafness with a sister and younger brother who are similarly afflicted. Renal biopsy Teaching Notes for Question 42 Theme: Alport's syndrome Only renal biopsy can confirm a diagnosis of Alport's.PEDIATRICS EXAMINATION REVIEW 41-Choose the incorrect statement. ear and eye leading to clinical disease of haematuria. Audiometry E.

C. Collapse right lower lobe C. outlines liver due to perforation of an intraperitoneal 175 . Perforation E. Steroids are the agents of first choice. Bilateral pneumothoraces Teaching Notes for Question 44 Theme: Chest X-ray . and can be administered via the oral.PEDIATRICS EXAMINATION REVIEW A. Consolidation right lower lobe B. Iridocyclitis usually responds to topical steroids. B. Hydroxychloroquine is associated with ocular toxicity. E. Gold salts may lead to nephrotic syndrome. In cases of oligoarticular disease. pulmonary fibrosis and GI symptoms especially nausea and vomiting. intra-articular steroids are usually tried first before systemic treatments. Side Effects include marrow suppression. D. 44-What is the abnormality shown on Chest X-ray? Options: A.Perforation Perforation * Free air under the hemi-diaphragm. Collapse lingular lobe D. Popular viva question in Part2: MTX is given weekly. subcutaneous or intramuscular route. Low dose methotrexate may be beneficial Teaching Notes for Question 43 Theme: treatment of Juvenile Idiopathic Arthritis Methotrexate is the treatment of choice in polyarticular and extended oligoarticular diseases.

Proliferative DM retinopathy B.PEDIATRICS EXAMINATION REVIEW viscus (not a retroperitoneal viscus). The haemorrhages and exudates look too well defined for this to be CMV retinitis. This slide demonstrates retinal blot haemorrhages and exudates. 45-What is the diagnosis? Options: A. Endocarditis would tend to produce more discreet white balls on fundoscopy. E. Background retinopathy Teaching Notes for Question 45 Theme: Retinopathy. Is more common in women than in men. Requires treatment with desferrioxamine to prevent iron overload. Is associated with haemolytic crises induced by sulfonamides. Is caused by mutations of the G6PD gene on chromosome 4. The absence of new blood vessels is against a proliferative retinopathy. C. CMV retinitis E. Often leads to chronic haemolysis. Grade 3 hypertensive retinopathy C. The absence of microaneurysms is against a diagnosis of background retinopathy. Teaching Notes for Question 46 Theme: Glucose-6-phosphate dehydrogenase deficiency 176 . 46-All of the following statements about Glucose-6-phosphate dehydrogenase deficiency are false except: Options: A. This appearance would do for hypertensive or diabetic retinopathy. B. D. Endocarditis D.

Anti-liver/kidney microsomal antibodies (anti LKM-1) in type I autoimmune hepatitis B. If severe. Reduced levels of C3 can also be seen in cholesterol emboli. certain drugs . C4) leads to bacterial infections. Anti-liver cytosol antibodies in primary sclerosing cholangitis 177 . G6PDH enzyme deficiency. CD59 or DAF deficiencies result in paroxysmal nocturnal haemoglobinuria. chemicals . Deficiencies of MBL (MBL. The NADPH is then used to reduce glutathione and hence in conjunction with glutathione reductase reduces cellular oxidative damage. Anti-phospholipid antibodies in Primary biliary cirrhosis C. caused by mutations in the X-linked (Xq28) G6PD gene. MASP2.usually the deficiency manifests as acute haemolytic crises provoked by oxidising stress e. G6PD deficiency can cause immunodeficiency by reducing the NADPH required for activation of the NADPH oxidase enzyme (cf. Anti-mitochondrial antibodies in Primary biliary cirrhosis D. C2.g. nitrofurantoin. ANCA in type II autoimmune hepatitis E. Haemolytic crises vary in severity. The various mutations lead to altered expression of G6PD or an altered half-life of the enzyme.patients may present with neonatal jaundice . sulphonamides.fava bean. primaquine. rarely chronic haemolysis may occur. This evolutionary advantage outweighs the small negative effect of affected hemizygous males. C3 def is associated with partial lipodystrophy E. There are a number of disease manifestations: . CGD). mainly in childhood. vitamin K and nalidixic acid.000 fold!). C1q deficiency and angio-oedema C. P) leads to infection with pyogenic bacteria and Neisseria but no immune complex disease. 47-Which of the following statements correctly describes the clinical phenotypes which are associated with a deficiency of the various complement components? Options: A. Infections can also provoke haemolysis. chloroquine. C4) predispose to immune-complex disease and systemic lupus erythematosis (due to inefficient removal of apoptotic cells). affects over half a billion people worldwide.C2. aspirin (high doses). 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 the classical pathway (C1. post streptococcal glomerulonephritis and mesangiocapillary glomerulonephritis type II. both C3 and C4 are reduced. Deficiencies in the alternative pathway (Factor D. C5-C9 deficiency and mycoplasma infection B. 48-The following autoantibodies are commonly found in the named disease: Options A.dapsone. It is a balanced polymorphism associated with resistance to falciparum malaria in heterozygous females.patients may be asymptomatic . Hereditary angioneurotic oedema is due to C1 inhibitor deficiency. At least 400 G6PD variants have been identified. certain foods . MASP1. Deficiencies in the components of the membrane attack complex (C5-C9) result in susceptibility to Neisseria infection only (estimated 10.naphthalene. C2 deficiency and rheumatoid arthritis D. C3 deficiency is associated with a wide range of pyogenic infections and Neisseria infections and sometimes with immune complex disease.PEDIATRICS EXAMINATION REVIEW G6PDH catalyses the synthesis of NADPH from the hexose monophosphate pathway. In active SLE.

pupils. 50-A two-year-old boy is brought to the resuscitation room. Soft aortic component of second heart sound D. severe MR/AR. The next step should be: Options: A. Continue to try for IV access and then give 20mL/kg fluid if successful B. 49-All of the following are clinical features of severe aortic stenosis except: Options: A. as always. Loudness of a murmur is usually NOT a clinical feature of its severity. Systolic thrill E. Type 2 is characterised by anti-liver/kidney microsomal antibodies (LKM-1). Secure intraosseus access and give 20mL/kg fluid D. His mother has noted that the left side of his face is dryer than normal. Where is the lesion 178 . On examination. anti-liver cytosol antibodies. 51-A patient is noted to have a small left pupil. Check a capillary blood gas Teaching Notes for Question 50 Theme: Resuscitation in sepsis The priorities. Check blood sugar C. and blood sugar). This child has a patent airway and adequate oxygenation. He has been unwell for 12 hours with fever and has vomited once. his airway is patent and he is breathing oxygen via a mask with non-rebreathing bag with oxygen saturations of 100%. 'disability' can be fully assessed (coma score.g. intraosseus access should be secured and fluid resuscitation with colloid administered. His heart rate is 160/min. and in a child like this who is unresponsive to pain. Primary biliary cirrhosis is characterised by anti-nuclear antibodies and anti-mitochondrial (M2) antibodies. anti-smooth muscle antibodies and anti-mitochondrial antibodies. Give immediate IM ceftriaxone before securing IV access E. After fluid resuscitation.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 48 Theme: Autoimmune hepatitis Type I autoimmune hepatitis is characterised by the presence of anti-nuclear antibodies. A colleague has been trying for 5 minutes to secure intravenous access when you arrive. He is not responsive to pain. It is very difficult to secure IV access when a child is so peripherally shut down. decompensated heart failure. capillary refill time is 5 seconds and he has a purpuric rash over his lower limbs. posture. There is also a ptosis present in this eye. Third heart sound C. Ceftriaxone can be given through the IO needle. 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. Fourth heart sound B. The current priority therefore is treatment of his shock (tachycardia and prolonged CRT). are ABC. Primary sclerosing cholangitis is characterised by ANCA positivity.

Immunosupressed people due to disease or treatment B.It is recommended for people who are at risk of serious influenza or its complications. and for people who can spread influenza to those at high risk 179 . 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. Cervical sympathetic trunk Teaching Notes for Question 51 Theme: Horner's syndrome Interruption of the cervical sympathetic trunk results in Horner’s syndrome. Lack of lacrimation Teaching Notes for Question 52 Theme: Horner's Symptoms of Horner’s syndrome include miosis. Causes include carotid pathology (tumours. lung lesions (especially apical squamous cell tumours). Trigeminal nerve C.PEDIATRICS EXAMINATION REVIEW likely to be? Options A. Vagus nerve E. 53-Which of the following sub-group of populations is the influenza vaccine not recommended for?:Options A. Those with diabetes mellitus E. anhydrosis and enophthalmos. It is due to an interruption in the symapthetic nerve supply to the eye. Those with chronic heart disease C. a syrinx and lung lesions. ptosis. Pupillary constriction B. Since these vaccines will not control an epidemic they are only for those people at high risk from infection. Possible causes include vascular lesions (carotid pathology). neck injuries. Absence of sweating on face and neck E. Ptosis C. Which of the following is NOT a symptom of Horner’s syndrome? Options: A. aneurysm and dissection). Oculomotor nerve B. Facial nerve D. Especially apical squamous cell carcinoma. A Horner's consists of ptosis. Every year the World Health Organisation (WHO) recommends which strains should be included. trauma and syringomyelia. 52-A patient is diagnosed as having a Horner's syndrome following carotid surgery. Those with chronic renal failure D. pupil constriction and absent sweating over the affected side of the face. Sinking in of one eye D.

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. Is often associated with an irregular pulse D. which usually cycles at a rate of 300 bpm (280–320). • People 65 years of age and older. Can be terminated with intravenous adenosine E.heart disease .01 and difference is found (p = 0.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). • Residents of long-term care facilities housing persons with chronic medical conditions. C would be correct if it said 0.metabolic disease. such as diabetes . 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. A larger sample should have been used B. Although usually regular.kidney disease . giving an irregular heart rate. there is good evidence to accept the alternative hypothesis. Other medical conditions. including coeliac disease are not high risk for developing influenza. every second flutter wave is conducted (2:1 AV block). Is usually left atrial in origin B.anemia. The ventricular rate is determined by the degree of AV nodal block.001).PEDIATRICS EXAMINATION REVIEW (including all household members): People at high risk for complications from influenza: • All children 6-23 months of age. 54-The lipid-lowering effect of a new statin is compared to atorvastatin. 180 . What is the best conclusion to draw? Options A.HIV/AIDS or other diseases affecting the immune system . • Women who will be pregnant during influenza season. • People who have long-term health problems with: . the degree of AV block sometimes varies from beat to beat. Usually causes a narrow complex tachycardia at 150 bpm C. The vaccine is an inactivated virus.1%. and other blood disorders • People with certain conditions (such as neuromuscular disorders) that can cause breathing problems.asthma . Critical level is set at 0. The p-value is the probability of committing a Type I error. • People with a weakened immune system due to: . The p-value associated with this statin means that it is a clinically significant new drug E. giving a ventricular rate of 150 bpm.lung disease . The probability of a type I error occuring is 1% D.long-term treatment with drugs such as steroids . The alternative hypothesis should be accepted C. Commonly. 55-Which of the following is correct regarding atrial flutter? Options: A.

which can be interrupted by radiofrequency ablation. TOF is associated with dextroposition of the aorta E. The chest X-ray shows oligaemic lung fields C.the subclavian artery is joined to the pulmonary artery -Waterston operation . Children with TOF are susceptible to cerebral abscess B.due to spasm of the infundibular muscle. The target HbA1c is about 8%.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. * Severity is related to the degree of right outflow tract obstruction.PEDIATRICS EXAMINATION REVIEW * Adenosine does not terminate atrial flutter. The second heart sound is soft and single D.usually not present at birth but develops over the first year. Patients present with: -Cyanosis . -'Hypercyanotic attacks' . During these attacks the infant becomes cyanosed. * Most atrial flutter originates in the RA. * The circuit of current usually occurs via an anatomically distinct pathway. * Pulmonary stenosis affects the pulmonary artery. 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. 57-In terms of the NICE guideline of screening for diabetic nephropathy in Type 2 patients: Options: A. Surgery is usually in two stages: -Blalock shunt . Loss of consciousness may occur. 181 .this increases systemic blood pressure. 56-The following statements are true about Tetralogy of Fallot (TOF) except: Options: A. restless and tachypnoeic. On auscultation. which can be diagnostic. the murmur may decrease in intensity -Squatting . This disorder results in a left to right shunt Teaching Notes for Question 56 Theme: Tetralogy of Fallot TOF. and increases pulmonary blood flow -Clubbing. pan or ejection systolic murmur due to right ventricular obstruction. 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. single second heart sound. with a counterclockwise circuit around the tricuspid valve annulus. but does increase the degree of AV block – this slows the ventricular rate transiently and may reveal flutter waves on ECG. the pulmonary valve and the infundibulum.the ascending aorta is joined to the right pulmonary artery Total repair is undertaken at school age.

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. C. has crept posteriorly) 182 .5% * BP target 135/75 * albumin:creatinine ratio >2. Left pneumothorax B. www. if no contraindications. Left tension pneumothorax E.5-7. one can check.nice. Calculating ratios of albumin and creatinine in the urine is not as useful as the total urine protein level. D.org. Blood pressure is less important than reducing the degree of proteinuria.uk In fact: * target HbA1c is 6. Left lingula collapse D. Left hilar adenopathy Teaching Notes for Question 58 Theme: Tension pneumothorax * Left hemithorax greater volume than right * Air outlines right mediastinal border (i.5 (men) and >3. All patients with confirmed microalbuminuria. Teaching Notes for Question 57 Theme: Diabetic nephropathy For this nephropathy and the retinopathy guideline. E. A renal consultation is advised if the serum creatinine rises above 250 µmol/l.PEDIATRICS EXAMINATION REVIEW B.e. should be on an ACE inhibitor. Left lower lobe collapse C.

Thymus C. skin is highly immunogenic. This membrane protein can bind to Fas on infiltrating leukocytes. Several factors may contribute to immune privilege. Teaching Notes for Question 60 Theme: Axillary nerve The deltoid atrophies when the axillary nerve (C5. a 10-year-old boy presents with a chronic cough. recurrent lung infections and clinical evidence of obstructive pulmonary disease. The precise mechanisms are not clear. Skin B. B. The rounded contour of the shoulder disappears. A loss of sensation may occur over the ‘epaulette’ area of the shoulder. cornea. skin grafting was used as a clinical test before solid organ transplantation to determine the tissue type compatibility between the donor and recipient. 60-Following a dislocated shoulder in a rugby match that gets out of hand. In contrast. and can induce apoptotic cell death in the Fas expressing cell.brain. His mother reveals that when he was 2 years old he was diagnosed as having ‘malabsorption’. The patient may have a wrist drop. 61-Steve. 5–10% of cases manifest with meconium ileus at birth B. and she has noticed that his sweat tastes excessively salty. D. A loss of sensation may occur in the lateral forearm. Testicle E. Pancreas Teaching Notes for Question 59 Theme: Immune privilege Some tissues fail to elicit an immune response and are termed immunologically privileged sites. Until modern HLA typing techniques developed. What is the likely result? Options: A. The eye also has high concentrations of TGF-beta which is a cytokine with immunosuppressive properties. The patient may lose the ability to adduct the arm. C. the rounded contour of the shoulder often disappears. the genetic abnormality is a four-base pair deletion at the locus on chromosome 7 183 . uterus and arguably liver. the existence of tolerogenic donor immature dendritic cells or their precursors within the liver graft. testis. Both the eye and the testis constitutively express Fas ligand. 6) is damaged. The blood-brain barrier may block entry of lymphocytes into this organ and the avascularity of the cornea may also limit antigen/lymphocyte interaction.PEDIATRICS EXAMINATION REVIEW 59-Which of the organs below is considered an immunologically privileged site :Options: A. In 70% of patients. Liver transplantation may induce antigen-specific tolerogenic effects. a patient’s axillary nerve is damaged. but have been attributed to the establishment of microchimerism. The teres major atrophies. Bone marrow D. All the above are features of this condition EXCEPT: Options: A. Therefore. E.

1 mmol/l 178 mmol/l 30 g/l 80 g/l Normal 7. blood 2+ proteins. and failure to thrive. The gene product is a cAMP regulated chloride transporter. * * * * * On examination she has no focal neurology. caused by a mutation in the gene encoding cystic fibrosis transmembrane conductance regulator (CFTR). Her blood test results reveal: Hb MCV WCC Platelets CRP ESR Sodium Potassium Urea Creatinine Albumin Total protein LFTs BM 9. Abnormalities in epithelial cell chloride and water transport result in viscid secretions in the respiratory tract. The CFTR gene is found in region q31-q32 on the long (q) arm of human chromosome 7. Viscid gastrointestinal secretions may result in meconium ileus at birth and in distal intestinal obstruction syndrome later in life. The disease incidence is about 1/2. Urinalysis tests 3+. Pancreatic insufficiency occurs in 75%–80% of patients. This deletion causes loss of the amino acid phenylalanine located at position 508 in the protein. Increased viscosity of bile with biliary ductule obstruction may ultimately result in cirrhosis. She smokes 20 cigarettes per day and drinks 15 units of alcohol per week. Recurrent inflammation and chronic bacterial colonisation with organisms such as staphylococcus aureus. bronchiectasis. therefore. mucoid form of pseudomonas aeroginosa and rare microbes such as burkholderia cepacia result in extensive pulmonary structural damage. There is evidence of lipid malabsorption.6C. pancreas. Pancreatic abnormalities are present in 80% of patients D.8 mmol Clotting screen: PTT APTT VDRL 11 seconds 39 seconds Positive 1:320 184 . Her only medication is the OCP. this mutation is referred to as deltaF508 CFTR. 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 Cystic fibrosis is an autosomal recessive disorder. 62-A 26-year-old airhostess is brought to the A&E department following a witnessed grand mal seizure. The primary defect is in the transport of chloride ions across epithelial tissue E. About 70% of mutations observed in CF patients result from deletion of three base pairs in CFTR’s nucleotide sequence.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.PEDIATRICS EXAMINATION REVIEW C.300 and the average life expectancy is about 31 years. Patients present with recurrent respiratory infections. Fundoscopy reveals silver wiring and AV nipping bilaterally. BP 160/98 mmHg. She has been previously fit and well except for a history of mild intermittent arthralgia affecting both hands. gastrointestinal tract. sweat glands. endstage lung disease and early death. There is slight puffiness of her fingers and she has low-grade pyrexia of 37. and other exocrine tissues.9 g/dl 83 fl 3.

Pulmonary embolism D. lateral. C. Epilepsy C. A lesion of this cord will cause total paralysis of the small muscles of the hand and have the same effect as a combined ulnar and radial nerve palsy. The medial cord supplies the medial branch of the median nerve and the ulnar nerve. AVN is a well-recognised complication of steroid treatment. prolonged APTT and PE are compatible with associated anti-phospholipid syndrome. This patient has central nervous system (CNS) and renal lupus.PEDIATRICS Drug screen of urine Plasma alcohol EXAMINATION REVIEW Negative Not detected What is the likely diagnosis? Options: A. 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. Kawasaki disease B. Infectious mononucleosis C. The C5 spinal root. The positive VDRL. Lesions of the lateral cord affect the musculocutaneous nerve and the lateral root of the median nerve. Syphilis E. 64-A boy presents with fever. The long thoracic nerve. The ulnar nerve. posterior and medial. Teaching Notes for Question 63 Theme: Brachial plexus injury The brachial plexus has three cords. This manifest as weakness of forearm flexion and pronation. which his mother says has been present for one week and has increased in size. B. arthralgia. Sensory loss would occur at the upper outer arm. arthralgia and headache and a red. Scarlet fever 185 . Posterior cord of the brachial plexus. The posterior cord supplies the deltoid muscle and all extensors of the arm and fingers. Infraclavicular injuries may damage the cords. The medial cord of the brachial plexus. SLE B. The probable site of the injection was the: Options: A. annular rash in his axilla. 63-An elderly man presents to casualty with a wrist drop and pain radiating down his right arm. Which is the most likely diagnosis? Options: A. and an elevated ESR with normal CRP is indicative of systemic lupus erythematosus (SLE). He recently visited his GP for a deep IM steroid injection. D. E.

myalgia. aseptic meningitis and chronic arthritis. and rarely encephalitis. Treatment is with doxycycline or amoxycillin or IM ceftriaxone if there is neurological involvement. central nervous system. pronator sign and darting motility of the tongue. Cat scratch fever Teaching Notes for Question 66 Theme: Most likely diagnosis of fever and jaundice. heart block. These include meningitis. Typhoid usually presents with fever. heart. but when present includes AV block. headache. Lyme disease is the most common tick-borne illness in the United States and occurs widely throughout Europe and the former Soviet Union. shoulder and elbow.32 days. muscle. Hemiballismus B. malaise.caused by the spirochaete Borrelia burgdorferi. The presence of jaundice would make which one of the following diagnoses more likely? Options: A. Cardiac disease is relatively uncommon in children. 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. joints etc.knee. arthralgia and malaise. Typhoid B. 15% of patients present with neurological symptoms and signs. regional lymphadenopathy. and it tends to enlarge over 1-2 weeks. Toxocariasis D. 65-Milkmaid grasp is characteristically seen in which of the following conditions? Options A. Diagnosis is confirmed by serology. Joint involvement is commonly asymmetrical and usually affects the large joints . Huntington’s disease D. Malaria C. cranioneuropathies. 66-A 2-year old boy is admitted with fever.PEDIATRICS EXAMINATION REVIEW D. Incubation period is 3 . The annular rash of Lyme disease (erythema chronicum migrans) is usually the first feature. headache and constipation. burgdorferi on the skin in faecal material. The Ixodid tick is harboured by deer in the New Forest. Rheumatic fever Teaching Notes for Question 64 Theme: Most likely diagnosis This is a case of Lyme disease . Generally seen in children. Arthritis is caused by persistence of organisms in the synovium. The vector is the Ixodid species of tick. Features include VII palsy. 186 . Torticollis E. Lyme disease E. uveitis. complete heart block and myopericarditis. Late disease presents weeks to months after inoculation. encephalitis. Sydenham’s chorea C. in association with non-specific symptoms such as fever. especially peripheral seventh nerve (Bell palsy)(3%). this chorea has other clinical signs such as the spooning sign. eye. Transmission is by a tick bite or by deposition of B. The spirochaete may disseminate via the lymphatics or the bloodstream to the skin. Lyme disease E.

Toxocariasis (organism: the nematode Toxocara canis from dogs. oral polio (Sabin)). Chronic hepatitis B infection (high infectivity) 187 . He should have all immunisations except pertussis C. He should receive the oral pertussis vaccine when next immunised E. Chronic hepatitis B infection (low infectivity) D. Past hepatitis B infection C. arthralgia. Acute hepatitis B infection B. What will you recommend for this child in the future? Options A. He should receive the acellular pertussis vaccine when next immunised D. wheeze and eosinophilia caused by the immune response to migrating larvae.5C within 48 hours of pertussis vaccination * Seizures or encephalopathy within 72 hours of pertussis vaccination * Prolonged unresponsiveness or collapse * Anaphylaxis or bronchospasm * Inconsolable screaming for more than 4 hours In the case of a severe generalised reaction. Lyme disease (organism: Borrelia burgdorferi) usually presents with skin lesions (erythema chronicum migrans). 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). 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.PEDIATRICS EXAMINATION REVIEW 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. He should not have any further immunisations B. Any oral vaccine will be a live vaccine (e. the acellular pertussis vaccine should be administered the next time. CNS or joints. On examination. There is no oral pertussis vaccine. 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'). * 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. In the case of a severe local reaction. fever. A severe generalised reaction is defined as any of the following: * A temperature >39. hepatomegaly. Cat scratch fever (organism: gram negative bacillus Bartonella henselae) presents with (usually cervical) lymphadenopathy and a history of a cat scratch. 67-A two-month-old child had his first DTP injection 3 days previously and his mother has returned because his thigh is swollen. and headache. diphtheria and tetanus ONLY should be given next time. most of the anterolateral surface of the thigh is red and indurated. Disease can localise to heart.g. oral typhoid. or Toxocara catis from cats) presents in one of 2 forms: * Visceral larva migrans is a syndrome of fever.

Babies born to mothers with high infectivity require passive and active immunisation (HBIg and vaccine). MDMA Teaching Notes for Question 70 Theme: Acidosis Drugs causing high anion gap acidosis include salicylates. Pco2 4. Causes include tumours secreting ADH (small cell lung cancer. lymphoma). 70-A 14 year-old boy is seen in the Ambulance and Emergency Department. Cl 110. prostate. The 'e' antigen status determines the infectivity of the carrier. serum levels of Na.29 . Glue E. and paracetamol. K. the fact that she is IgG positive suggests she has chronic infection. plasma protein levels are normal. Methanol B. 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). so dependent oedema is rare. including Guillain-Barré syndrome. ‘e’-antigen positive (but antibody negative) means high infectivity. or to not yet have formed antibodies to the core antigen. Urine osmolality is >500 mosm/kg E. Opiates D. Therefore. lung infections. Ethylene glycol C. and acute intermittent porphyria (AIP). pH 7. The following blood test results are found. methanol. He is unconscious and he has a rash around his mouth. pancreas. ethylene glycol. urea. thymus. K 5. CNS injury. Urine is inappropriately concentrated. Na 135. signifies low infectivity. HCO3 15. drugs. This has relevance if this girl becomes pregnant. If this was acute hepatitis B infection one would expect her to be IgM positive. Impaired hepatic excretion of lactate. creatinine. which will always be found in people who have had hepatitis B infection (either currently or in the past). Oedema may occur due to fluid overload D. 69-In the syndrome of inappropriate ADH secretion (SIADH): Options: A. or conversion to acids metabolically 188 . What has he taken? Options: A. and urate are all lowered. She has antibodies to the core antigen. Plasma urate levels are elevated C. However. antibodies to the ‘e’ antigen (but no ‘e’ antigen detected) as in this case.5. Serum globulins are high Teaching Notes for Question 69 Theme: Syndrome of inappropriate ADH secretion (SIADH) SIADH produces inappropriate water retention despite low plasma osmolality. babies born to mothers with low infectivity require vaccination alone. Plasma sodium is usually normal B.PEDIATRICS EXAMINATION REVIEW E.

Variegate porphyria is associated with pigmentation. D. 72-Acute attacks of porphyria:Options: A. Abdominal pain. On examination. He reports this at a distance of 3 m and also at 6 m. he reports having only central vision over an area of approximately 1 m by 1 m. diarrhoea. Kidney failure. Can produce hyponatraemia due to Diabetes Insipidus C. Splenic rupture. Teaching Notes for Question 71 Theme: Infectious mononucleosis The spleen is enlarged and soft in consistency in this disease. C. Can be precipitated by paracetamol B. “Non-organic” symptoms E. aspirin and opiates are safe analgesics in porphyria. If care is not taken while palpating the spleen. acetazolamide. Malabsorption is not a feature of porphyria. Heart failure. it may rupture and this is fatal in 25% of cases of infectious mononucleosis. Drugs causing normal anion gap acidosis include amphotericin. 73-A 17-yr-old male medical student presents with a sudden onset of tunnel vision. Congenital porphyria (CP) and Erythropoetic Protoporphyria (EPP). and solvents (toluene) *glue*. therefore a hyperchloremic acidosis with a normal anion gap. Optic neuritis B. Are a feature of porphyria cutanea tarda D.PEDIATRICS EXAMINATION REVIEW can also result in acidosis with high anion gap. Are precipitated by the contraceptive pill E. Methanol poisoning D. 71-The most important cause of death in infectious mononucleosis is: Options: A. Which of the following diagnoses best fits his symptoms? Options: A. Are associated with malabsorption Teaching Notes for Question 72 Theme: Porphyria Acute attacks do not occur with Porphyria cutanea tarda (PCT). Retinitis pigmentosa 189 . and nausea can all occur in acute attacks. E. Anterior uveitis C. B. Paracetamol. Hereditary coproporphyria (HCP) and Variegate porphyria (VP) have skin manifestations and acute attacks. Another cause of normal anion gap acidosis is renal tubular acidosis. constipation. Porphyria rarely presents before puberty and occasionally first presents with an acute acttack triggered by the OCP. hirsuitism and blisters as well as acute attacks. AIP is the only one without skin manifestations. Glue poisoning usually gives rise to a distal (type 1) RTA. Liver failure. Respiratory failure.

PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 73 Theme: Visual fields Tunnel vision should show an increasing area of vision with distance. T1 spinal root D. Ulnar nerve Teaching Notes for Question 74 Theme: Brachial plexus lesions Wrist drop is indicative of a process affecting the radial nerve. right arm and shoulder during a game of tennis.1 mmol/l 10. Medial cord of the brachial plexus C. On examination she has no focal neurology. 2+ protein. progressing to visual field contraction and sometimes complete blindness. This arises from the posterior cord of the brachial plexus. Anterior uveitis tends to cause pain and blurred vision. 75-A 26-year-old air hostess is brought to A&E following a witnessed grand mal seizure. Retinitis pigmentosa will cause symptoms of tunnel vision.9 g/dl 83 fl 3. Her only medication is the OCP. 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. Posterior cord of the brachial plexus B. BP 160/98 mmHg. Optic neuritis will lead to blurring and central scotomas. Urine analysis tests show 3+ blood. she smokes 20/day and drinks 15 units of alcohol per week.6°C.1 X 109/l 117 X 109/l 2 g/l 71 mm in the first hour 134 mmol/l 5. A fixed area implies non-organic symptomatology. The ulnar nerve innervates the small muscles of the hand while the medial cord gives rise to the median nerve. Methanol poisoning begins with blurred vision. She has been previously fit and well except for a history of mild intermittent arthralgia affecting both hands. Fundoscopy reveals silver wiring and AV nipping bilaterally. 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 9.1 mmol/l 178 mmol/l 30 g/l 80 g/l normal 7. C5 spinal root E. The probable site of the injury was the:Options: A. Accidental injection of spinal roots seems unlikely and would not cause a complete palsy.8 mmol 11 seconds 39 seconds positive 1:320 negative not detected 190 . There is slight puffiness of the fingers and low-grade pyrexia of 37.

diarrhoea. Which is the next investigation to confirm the diagnosis? Options: A. 0. O2 sats 94% on room air. 77-A 26-year-old woman is admitted to hospital for cadaveric renal transplantation. but at an OPD appointment 3 months later is complaining of worsening right hip pain poorly responsive to NSAIDs. Avascular necrosis C. High-dose dexamethasone test is a valuable outpatient screening test. whereas the low dose dexamethasone test is conducted while the patient is admitted in the hospital. Stress fracture E. 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. arthralgia. The positive VDRL. If it is not suppressed then the diagnosis of Cushing’s syndrome is made after taking the urinary cortisol results into account. AVN is a well-recognised complication of steroid treatment. This patient has CNS and renal lupus. Trochanteric bursitis Teaching Notes for Question 75 Theme: SLE. weight gain and striae is being worked up for Cushing’s syndrome. central obesity with striae. High-dose dexamethasone suppression test C. skin fragility. Osteoporosis B. Septic arthritis D. however 10 days after admission she develops a fever. hirsuitism. * Cushing’s disease is the cause of Cushing’s syndrome in around 60% of cases. BP 105/55. Measuring cortisol levels D. * Commonest clinical features of Cushing’s include hypertension. She is given treatment with prednisolone and cyclosporin.PEDIATRICS EXAMINATION REVIEW The patient then develops acute dyspnoea and examination reveals decreased air entry at the right base. prolonged APTT and PE is compatible with associated anti-phospholipid syndrome. Low-dose dexamethasone suppression test B. The operation is uneventful however she is admitted to the Intensive Therapy unit for 48 hours postoperatively. 191 . diabetes mellitus and muscle wasting. She responds well to prednisolone and azathioprine. those with surgically amenable lesions tend to do well. adrenal neoplasms are responsible for around 30%. emotional lability.Complications The presentation with a fit in the absence of structural or metabolic derangement accompanied with pancytopenia. What is the most likely cause of her hip pain? Options: A. X-ray is normal but bone scan shows increased uptake in the hip. Her 24-hour urinary free cortisol is 450 nmol. 76-A 35-year-old woman who complained of easy bruising.5 mg of dexamethasone is given every 6 hours till 8 doses are completed and suppression results are noticed. * Prognosis is poorest in patients where the cause is malignant adrenal neoplastic disease. and ectopic ACTH production is responsible for the remaining 10%. tachycardia. Measuring ACTH levels E. and an elevated ESR with normal CRP is indicative of SLE. headaches and a non-productive cough. osteoporosis.

She is treated with cefotaxime. This raises the possibility of atypical organisms. however after 48 hours the fevers continue and a repeat CXR shows left lower lobe consolidation. Klebsiella pneumoniae C.PEDIATRICS EXAMINATION REVIEW Examination reveals no focal signs of infection. they should be considered as a cause of invasive infections. Cytomegalovirus E. Options: A. CMV and C. sodium bicarbonate or hypertonic saline would also not address the basic electrolyte imbalance. particularly in individuals who had been nursed in renal and high dependency units. iv isolyte – P C. Although coliforms are frequently isolated from the sputum of hospitalised patients. Cryptococcus neoformans Teaching Notes for Question 77 Theme: Nosocomial pneumonia Although Streptococcus pneumoniae should be considered as one of the most likely causes of postoperative pneumonia in an immunosuppressed patient. resistance to 3rd generation cephalosporins is common in the noscomial setting. hypokalaemic. a response to Cefotaxime would be expected within 48 hours. Infusion of 5% dextrose. iv sodium bicarbonate E. The dehydration may result in hypernatraemia or hyponatraemia and there may be pre-renal renal failure. Metabolic alkalosis causes a decrease in ionised calcium because of increased binding of calcium to plasma proteins. metabolic alkalosis is the classic electrolyte and acid-base imbalance associated with the non-bilious vomiting of pyloric stenosis. however this soon after a transplant P. Hypochloraemic.)Dyselectrolytaemia 2. Two major diagnostic considerations are:1. The intravenous (iv) fluid of choice for administration would be: Options: A. Obviously serum electrolytes will be measured. He is dehydrated and afebrile with a depressed anterior fontanelle (AF). Pneumocystis carinii D. 192 . 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. Infusion of calcium gluconate would not correct the metabolic abnormality. Blood cultures are taken and a CXR is unremarkable.)Septicaemia and meningitis A septic screen should be performed and antibiotics commenced. iv 50% calcium gluconate B. Streptococcus pneumoniae B. iv 5% dextrose D. neoformans are very unlikely. He has been vomiting non-billious breast milk for the last 3 weeks. Ultrasound of the abdomen shows hypertrophic pyloric stenosis. 78-A 6 week old male baby presents with a 2 day history of multiple seizures. On examination the child is afebrile and dehydrated with a depressed anterior fontanelle. Intravenous (iv) Isolyte P consists of 5% dextrose with Na. K and Cl supplementation. particularly those who may have had many previous courses of antibiotics. Replacement should be guided by the clinical response and the electrolyte balance. carinii. and is used in the treatment of paediatric dehydration.

Therapy is started and 5 days later he becomes acutely jaundiced with red discolouration of the urine. Penicillin C. It is a relatively common cause of community-acquired and nosocomial pneumonia in adults much less common in children. For example. HIV antibody test 193 . CXR shows hazy shadowing in the right mid and lower zones. Blood cultures E. IgM for viral haemorrhagic fever C. the incidence of diarrhoea is similar in Legionnaire's disease. no association with underlying disease and zero mortality. pneumococcal pneumonia and mycoplasma pneumonia. a 16-year-old boy develops fever and rigors. However cerebellar ataxia. 80-A 15 year old boy is admitted with pneumonia. rifampicin should be given with erythromycin as when given alone it tends to increase resistance. Pontiac fever is a 'flu-like' illness that is more common than fullblown Legionnaire's disease. Which of the following drugs is the likely cause :Options: A. Preexisting immunocompromise is a risk factor C. Pontiac fever is a rarer and milder form of the disease E. Rifampicin alone increases resistance and is not as effective as 3 weeks of erythromycin. epidemic tendency. and patient should be warned that secretions will turn red (particularly tears and urine). Rifampicin alone is as effective as erythromycin for the treatment D. Rifampicin D. Clinical differentiation from the other causes of community acquired pneumonia is not possible.PEDIATRICS EXAMINATION REVIEW 79-Regarding Legionnaire's disease:Options: A. For Legionnaires' disease. Investigations show hyponatraemia and mildly deranged LFTs. 81-Seven weeks after returning from rural Ghana. has a shorter duration. affects younger patients. Diagnosis can be made from stool culture Teaching Notes for Question 79 Theme: Legionnaire's disease Legionnaire's disease is usually due to infection with Legionella pneumophila type 1. Ciprofloxacin E. Rifampicin is a hepatic enzyme inducer and can lead to acute jaundice. Chest radiograph D. pre-existing immunocompromise and COPD are risk factors. Strict barrier nursing is essential to contain the infectious agent B. Cefotaxime B. Tetracycline Teaching Notes for Question 80 Theme: Drug induced jaundice (1) The patient was started on rifampicin for suspected Legionella pneumonia. high alcohol intake. Male sex. smoking. Thick and thin blood film B. peripheral neuropathy and poor memory are recognised neurological complications and are probably mediated by a cerebral toxin (in addition to the effects of hyponatraemia). The least useful test is: Options: A.

PEDIATRICS

EXAMINATION REVIEW

Teaching Notes for Question 81
Theme: Fever in a returning traveller
Malaria is the most likely tropical diagnosis; therefore, multiple thick and thin blood films
are essential.
CXR and blood cultures are needed to rule out a respiratory infection or systemic sepsis.
Viral haemorrhagic fever should be considered in the differential diagnosis if the patient
has returned from an endemic area within the last 21 days; 7 weeks is too long, the
incubation period is 2–21 days.
82-A 15-year-old is undergoing investigation for hirsuitism. Which of the following would
not be implicated in the aetiology?
Options:
A. Cyclosporin A.
B. Phenytoin.
C. Moxonidine.
D. Congenital adrenal hyperplasia.
E. Cushing's syndrome.

Teaching Notes for Question 82
Theme: Hirsuitism
Drugs causing hirsuitism include cyclosporin/phenytoin/minoxidil (not moxonidine -a
centrally acting anti-hypertensive).
Polycystic ovaries and congenital adrenal hyperplasia are associated with increased
androgens and hirsuitism.
Treatment is with anti-androgens (cyproterone, spironolactone), reducing free androgens
(oestrogen therapy, OCP, weight loss), and cosmetic.
83-A family attends genetic counselling. They have one child affected with Down’s syndrome
and are considering another pregnancy.
Which of the following statements is most accurate?
Options:
A. Both parents should have karyotype analysis, but there is no need to karyotye the affected child
B. 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. A karyotype of 46XX excludes a diagnosis of Down’s syndrome
E. 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, some will occur because of a balanced chromosomal translocation in one
of the parents. The parents in these cases are phenotypically normal and have a complete
complement of genetic material but the chromosomal arrangement is different. Because of
this, they have a high recurrence risk for other children to be affected with Down’s
syndrome. Consequently, all families with a child with Down’s syndrome should have basic
chromosomal investigations performed.
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, most children with Down’s syndrome are
born to younger mothers.
The triple test consists of measurements of alpha fetoprotein, bHCG and unconjugated
estriol. It will identify about 65–70% of those at high risk of carrying an infant with Down’s
syndrome.

194

PEDIATRICS

EXAMINATION REVIEW

The nuchal scan together with bHCG and PAP identifies those at high risk of a Down’s
syndrome pregnancy in >80% of cases.
84-A 17 year old student develops a deterioration in vision in her left eye over three days.
She complains of discomfort in the eye and thinks that difficulty with perception of colour
was the first problem that she noticed, during a trip to an art gallery. On examination, visual
acuity on the left is down to light perception. The pupil appears dilated and does not
constrict to light, although does when a torch is shone in the right eye.
What is the most likely diagnosis :Options:
A. CMV retinitis
B. Optic neuritis
C. Anterior iscaemic optic neuropathy
D. Amaurosis Fugax
E. 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, jaw claudication, scalp tenderness. High
ESR. Usually affects both eyes.
Central Retinal Artery Occlusion – Acute, with maximal deficit at the onset and variable
resolution.
Red spot at macula. Amaurosis Fugax – Transient blindness lasting minutes to hours.
Associated with vascular risk factors as usually embolic. May be described as a “curtain
coming down”Anterior
Iscaemic Optic Neuropathy – Painless, may be central field defect with colour disturbance a
prominent feature. Arteriosclerosis, hypertension and diabetes are risk factors.
Optic Neuritis – Painful, colour vision affected. May not result in complete visual loss. May
be the first manifestation of MS, therefore requires appropriate investigation.
Infectious Causes – CMV, Toxoplasmosis in association with immunodeficiency
85-A 29 year old Cypriot man gives a history of recurrent pain and swelling of the left knee.
He also describes recurrent episodes of fever, pleuritic pain, and rash. He is then
hospitalised with severe headache, photophobia and neck stiffness. He is lucid with a GCS of
15.
His blood test results show:
Hb 13.8g/dl
plats 210 X 109/l
WCC 4.9 X 109/l
plasma glucose 6.5 mmol/l
CRP 39g/l
ESR 56 mm in the first hour
RF, ANA, ENA and ANCA negative
CSF analysis:
opening pressure 14cmH2O
protein 0.6 g/l
RBC 2 /ml
WBC 21/ml (100% lymphocytes)
CSF glucose 4.1 mmol/l
no organisms seen on microscopy
48 hour culture of CSF negative
What is the diagnosis?
Options:
A. Familial Mediterranean fever

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B. Adult onset Still's disease
C. Behcet’s syndrome
D. Familial Hibernian fever
E. 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. Aseptic meningitis is a recognised clinical manifestation of FMF. The
splenomegaly and proteinuria is due to the development of amyloidosis, not an infrequent
consequence of untreated FMF.
Amyloidosis can complicate any condition characterised by a prolonged persistent acute
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.

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

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Is inherited as an autosomial dominant trait D. * MCNS is a histological diagnosis – the term steroid sensitive NS is used if a clinical diagnosis is made.Relapse on Prednisolone >0. Minimal change disease is a histological diagnosis Teaching Notes for Question 90 Theme: Nephrotic Syndrome * By definition this is “minimal change”. Hypervolaemia is a common problem C. May result in retinal abnormalities E.20% albumin is given for severe oedema.5% albumin is given if the child is severely hypovolaemic (as resuscitation fluid). gene is on chromosome 13. Kaiser Fleisher rings appears at the periphery of the iris. * These children are far more likely to be hypovolaemic.(LOAF). Albumin is routinely given to children with proteinuria D. which of the following statements is true :Options: A. especially tense ascites. then slow reduction 4.Relapse on Prednisolone > 0. The other muscles of the hand are innervated by the ulnar nerve. When penicillamine is administrated then urinary copper level rise C. Autosomal recessive inheritance. deposition is seen along the glomerular basement membrane B. 89-In a patient that has been previously diagnosed with Wilson’s disease. 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. followed by 40 mg/m2 on alternate days for 4 wks 2. 90-Which of the following statement(s) regarding childhood minimal-change nephrotic syndrome is/are true?:Options. Long-term cyclophosphamide therapy is beneficial in the steroid resistant group E. and * flexor pollicis brevis .5 mg/kg/ alternate days Levamisole 2. only Electron microscopy changes in the glomerular basement mambrane are seen in the form of increased spaces between the podocytes or foot processes.5 mg /kg/alt days with steroid side effects or >1.1-0.PEDIATRICS EXAMINATION REVIEW * abductor pollicis brevis. This makes the glomerulus more permeable and albuminuria occurs. Serum cerruloplasmin is low.0 mg/kg/alt days cyclophosphamide 2-3 mg/kg/day for 8 weeks 198 . 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. * 4. Serum cerruloplasmin levels are raised B.First two relapses: Prednisolone 60mg/m2/day (max 80 mg/day) until remission followed by 40mg/m2 on alternate days for 4 weeks 3. On immunofluorescent studies.Frequent relapser: Maintenance Prednisolone 0. scrotal oedema.5 mg/ kg /alternate days 5.Initial episode: Prednisolone 60 mg/m2/day (max 80 mg/day) x 4-6 weeks.5 mg/kg/alt days for 3-6 months. * Cyclophosphamide is beneficial but only as a short-term course over 8 weeks. PROTOCOL FOR MANAGEMENT OF NEPHROTIC SYNDROME: 1. A.

Every individual has two copies of over 40.chicken pox.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.3). low urinary Na (< 10 mmol/l) Thrombosis and embolism •Hypercoagulability due to increased plasma fibrinogen. BP may be normal . poor cap refill. cyclosporin) 91-A child has had his chromosomes analysed in the course of various investigations and the result is: 46 XY.p21. •· Bone marrow suppression (cyclophasphamide. There is a translocation between the short arm of chromosome 2 and the short arm of chromosome 5 D. thrombocytosis. increased toe core temp gap. An individual with a balanced translocation such as this is likely to be asymptomatic but is at risk of having a child with difficulties. diuretics Hypocalcaemia. 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). The karyotype 46 XY.due to loss of vitamin D binding protein in urine(also albumin loss. It does not describe an individual's genetic defects. •Septicaemia (gr negative ). oliguria.5)(q35. It is usually transmitted by rat bite 199 . 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. He is likely to be infertile E. cataract.PEDIATRICS EXAMINATION REVIEW 6.steroid(5% mortality). Which of the following statements is correct?:Options: A. It will accurately describe visible abnormalities in chromosome number and arrangements such as translocations.Post . t (2. Y chromosome abnormalities are much more likely to result in infertility.3}. factor V & VII by liver.high (reactive HT) or low •Raised Hb/Hct.need corrected Ca++ Complications of treatment•· Cushingoid effects.abdo pain. deletions and duplications. t (2. peritonitis(pneumococcal). measles Hypovolaemia. 92-In leptospirosis which one of the following is true? Options: A. He has more than 46 chromosomes B. oedema & reduced mobility.3) describes a male with a normal number of chromosomes but a translocation between the long arm of chromosome 2 (q) (sub band 35) and the short arm of chromosome 5 (p) (sub band 21. The result shows all his genetic defects C.p21. cold extremities.5)(q35.000 genes and many of these will have mutations that are of no consequence to the individual. decreased antithrombin III (lost in urine) + hypovolaemia. poor growth.

C. -Large salt & water loss-polyuria +polydipsia. Options: A.5. The patient may be jaundiced during the immune phase D. Nephrocalcinosis.PEDIATRICS EXAMINATION REVIEW B. -Generalized aminoaciduria (Tubular leak of all low molecular weight proteins).Total numbers of anions. PH typically > 6. -Impaired h+ secretion in distal tubule can not produce acid urine. (B2 microglobulins. Weil’s disease is the eponym for the most severe presentation with shock and multi-organ failure. In a small number of cases the disease presents as a septic illness 1–2 weeks after exposure. 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. -Rx: large amounts of Nahco3 needed. D. Her parents tell you that she has always been a very thirsty child. 200 . bicarbonate wasting.renal tubular acidosis -Potassium wasting with hypokalaemia. * The organism. don't get hypercalciuria or nephrocalcinosis. Cataract. B. * Treatment options are IV penicillin/amoxicillin for 7 days in severely ill patients or doxycyline for milder cases. and having soaking wet napping. Metabolic acidosis with a wide anion gap. nephrocalcionosis.RBP). = (Na+ K ) – (Cl +Hco3). -Once plasma bicarbonate falls low enough urine PH < 5. and 1+protein. Hyperkalaemia. Leptospira interrogans can be isolated from the blood or CSF during the septicaemic phase and from the urine during the 2nd week of illness. *Distal (classical type). K wasting and tubular proteinuria. NAG. drinking 3-4 bottles of water through the night. Renal tubular acidosis. urine dipstick shows 1+ glucose.Theme: Renal tubular acidosis . hypercalciuria.This is proximal renal tubular acidosis Anion gap = Total number of cations in blood . E. Which of the following features are you likkely to find on examination and investigation. Fanconi syndrome Causes. -Can excrete acid in distal tubulee. * Most patients suffer only a mild fever headache and muscle pains and the condition often goes undiagnosed. Teaching Notes for Question 93 . -Aldosteron deficiency. -Rx:small amount NaHco3 Hyperkalemic (TypeIV). Normal value = 8-12 Biochemical features: *Renal glycosuria. Corneal opacities. reduced phosphate resorption. Fanconi syndrome. -Pseudohypoaldosteronism. 93-A 9 months old baby girl is brought to you with failure to thrive and rickets. The organism may be isolated from urine during the septic phase E.generalised aminoaciduria . -Bicarbonate wasting due to low threshold. It is a notifiable disease C. -bicarbonate wasting. *Proximal (Type II). -Lwo Po4 bsorption-Hypophosphataemic rickets. -Renal glycosuria.

B. Which is the most appropriate initial course of action ? Options: A. all mostly given as outpatients . C. D.corneal deposits. Do a lymph node biopsy to exclude concomitant lymphoma. -Treatment . -Idiopathic. -Low’s syndrome (Oculo-Cerebral.diabetes.Renal syndrome). Osteoporosis. Discharge him and review in 1 week time as an out patient. Peripheral neuropathy. D. -Tyrosinaemia.impaired function. Tumoour lysis is common on induction of remossion and best prevented with IV fluids & allopurinol. Poor prognostic factors include: Age (<1. Cystinosis.large group. Start IV fluids & allopurinol. -Drugs. -Hereditary fructosaemia. hypodiploidy and MLL gene rearrangements. -Autosomal recessive.Which of the following is not a recognised complication of anticoagulation therapy? Options: A.esp amphetericin. Phosphocyteamic. -All tissues in body involved.unable to transport cystine out of lysosome. Deep venous thrombosis.PEDIATRICS EXAMINATION REVIEW -Cystinosis. Wbc cystine level. -Wilson’s disease.esp 1 fosphamide. C. Treatment. but there are small blasts in the blood film. Teaching Notes for Question 94 Theme: Acute lymphoblastic leukaemia Acute lymphoblastic leukaemia is commoner in children. lasts for 3 years in boys & 2 years in girls and involves blocks of maintenance chemotherapy and intensive chemotherapy. E. slit lamp. 95. -Antenatal diagnosis available. -Galactosaemia. *Kidney-proximal renal tubular acidosis. -Diagnosis – clinical suspicion. *Pancreas. Commonce chemotherapy straight away. -Heavy metal poisoning. *Liver. male sex. 94-A 5 years old presents to casuality with a 2 months history of inguinal lymphadenopathy. Increase cystine-transport out of lysosome. *Eyes. it generally presents with nonspecific symptoms but it is not uncommon to find lymphadenopathy on presentation . 201 . Thrombocytopaenia. his full blood count is normal . Skin necrosis. a bone marrow aspirate confirm that he has acute lymphoblastic leukaemia. E. B. >10). Referre him for a bone marrow transplantation. -Chemotherapy. -Lysosomal storage disorder. can delay onset of renal failure.

B. *The bladders of children with enuresis are smaller than other children. Long-term heparin therapy may be associated with osteoporosis. At 15 years age. Treatment involves stopping heparin and anticoagulating with hirudin or danaparoids. which one of the following statement(s) regarding enuresis is not true? Options: A. Haematologically normal. B-thalassaemia trait. he has no daytime enuresis. 96-The mother of a baby in the postnatal ward has an antenatal screening test for sickle haemoglobin.It is not possible for the baby to have sickle cell disease (HbSS) 97-A 9 years old male has always wet the bed. Although the main side effect of anticoagulation therapy is excessive bleeding.Sickle cell trait * .the father has no evidence of Hbs & has a . Runs in families. C. Her partner has a microcytic anaemia. The mother probably has a sickle cell trait. Warfarin. leading to sever thrombocytopaenia and occasionally catastrophic thrombosis.Normal Hb* . If a child has tried many treatments over a number of years the prognosis is poor.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 95 Theme: Anticoagulation. There is a 30% chance that his father was a bed-wetter. 202 . E. and Hb-electrophoresis shows an HbA band only with HbA2 quantitation of 4. more unusual side effect exist. may be associated with skin necrosis. B. C. Sickle cell diseas(HbSS). E.atelet antibodies form. Teaching Notes for Question 96 Theme: Haemoglobinopathy. Drinking tea causes bed-wetting D.Sickle-b-thalassaemia * . Heparin may be associated with both thrombocytopaenia and thrombosis as part of the disorder known as heparin-indused thrombocytopaenia (HITT). D.8%. Sickle-B-thalassaemia. This is positive deposite a normal Hb & MCV. Primary nocturnal enuresis. Teaching Notes for Question 97 Theme: Enuresis. there is a 1% chance that he will still bed-wet.laboratory & Hb-electrophoretic profile compatible with B0thalassaemia trait : The baby may therefore have . Sickle cell trait. This is an acquired immune disorder in which heparin-dependant anti-p. The Hb-electrophoresis shows HbA 55% and Hbs 45%.B-thalassaemia trait * . Some of the myths regarding enuresis are: *Medications are required in all cases. Which diagnosis is impossible in the baby? Options: A. *Children with enuresis wet the bed deliberately. especially induction therapy in patients with inherited protein C or S deficiency.

creatinin =141 . CSF examination shows markedly raised protein & a mild lymphocytosis. C. B. Sever weakness.20)C4= 0. B. IgA nephropathy (Berger’s disease) typically affects young adult males. in addition to old age. C.CH50= 87% (50-110) Urinalysis: blood 3+. Ig A nephropathy. Teaching Notes for Question 98 Theme: IgA nephropathy.20-0.551. on consulting his GP 3 days after the onset of this illness. Henoch-Schonlein purpura.1 . It is the site of most vitamin C absorption. 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. B. What is the most likely diagnosis? Options: A. D. Proteinuria rather than haematuria is more common in PSGN. D. D.4 urea=10. low grade fever and has some cervical lymphadenopathy. Markedly reduced FVC.Na=137 K=4.up to 10% of cases have sever residual disability. In arround 3% of cases death occurs . post-streptococcal glomerulonephritis.C3= 1. 10% develop renal failure. C. Mucous. presence of postural hypotension.42g/l (0. Teaching Notes for Question 99 Theme: Prognostic factors in Guillain Barre syndrome. Wegener’s granulomatosis. There is no history of antibiotics or NSAIDs for interstitial nephritis and no history of arthralgia or purpura for HSP 99-An adolescent male is admitted with progressive limb weakness. on examination he has flacid weakness predominantly in the legs and absent reflexes. investigations reveals: FBC normal .50).PEDIATRICS EXAMINATION REVIEW 98-A 18 years old student becomes un well with a sore throat. Denervation (rather thyan demyelination) show on nerve conduction studies implies a poor prognosis as do the remaining stems. few red cell casts. Interstitial nephrosis. History of diarrhea in the preceding few weeks. K and bicarbonate are absorbed. protein +. Evidence demyelination on nerve conduction studies. Which of the following does not imply a worse prognosis? Options: A. and immediately follows a viral infection or may have no clear precipitating factor. 203 .31g/l (0. Na and Cl are actively secreted. 100-Only one of the following is correct regarding physiology of the colon: Options: A. E. The diagnosis in Guillain Barre syndrome. E. Prognosis is good. examination is otherwise unremarkable.Stomach distension increases colonic motility.

PEDIATRICS

EXAMINATION REVIEW

E. Increased parasympathetic activity leads to decreased colonic motility.

Teaching Notes for Question 100
Theme: Colonic physiology.
Colonic functions include active absorption of Na and cl, and secretion of k, hco3 and
mucous. Parasympathetic activity, distension (gastrocolic reflex), emotional factors and
cholinergic agents increase colonic motility. Vitamin C is water-soluble (acscorbic acid) and
most absorption is proximal to the colon.

Questions
(PART 3)

SELECT THE MOST APPROPRIOT
DIAGNOSIS
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1-Options :
A. Osteomalacia
B. Ankylosing spondylitis
C. Avascular necrosis
D. Rheumatoid arthritis
E. Osteoarthritis
F. Paget’s disease
Instructions: Choose the most appropriate disease for the radiological description.
1. Narrowing of joint space, marginal osteophytes and subchondral cysts.
2. Bamboo spine
3. Mixed osteoblastic and osteolytic lesions in the left tibia. There is also cortical
thickening.
4. Joint space narrowing, juxtaarticular osteoporosis and bony erosions.
2-Options :
A. Streptococcal throat infection
B. Haemophilus influenza epiglottitis
C. Pneumococcal infection
D. Kawasaki’s disease
E. Typhus
F. Tuberculosis
G. Infectious mononucleosis
H. Erythema infectiosum
I. Mumps
J. Varicella zoster

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K. Rubella
L. Measles
Instructions: For each of the patients described below choose the single most likely option from the
above list. Each option may be used once, more than once or not at all.
1. A 5 year old girl presents with conjunctivitis, fever and coryza 12 days after exposure to a sick
sibling. On examination she has blue white punctate lesions on her buccal mucosa.
2. A 16 year old boy presents with fever, sore throat, anorexia, malaise. He has enlarged cervical
lymph nodes. On investigation there is lymphocytosis with atypical lymphocytes.
3. A 6 year old girl presents with fever and a slapped cheek erythematous eruption on her
cheeks.
4. A 8 year old boy presents with malaise, fever, and has painful swelling of both parotid glands.
5. A 6 year old boy presents with fever, cervical lymphadenopathy and erythema of palms and
soles.
3-Options :
A. Full blood count
B. Marrow biopsy
C. EEG
D. CT scan
E. INR
F. Thyroid function tests
G. Liver function tests
H. Liver biopsy
I. Prolactine levels
J. Drug serum levels
K. Breast ultrasound
Instructions: Drug side effects-investigations; give the most appropriate investigation for each
scenario. Each answer may be used once, more than once or not at all.
1. Patient on phenytoin, with diplopia, tremor, and low mood
2. Patient on Sodium Valproate, with vomiting, anorexia, jaundice, drowsiness
3. Patient on carbamazepine feeling unwell, with sore troat, and temp. 37.2 °C
4. Male patient on phenothiazines comes with discharge from his breast
4-Options
A. Down's syndrome
B. Turner's syndrome
C. Patau’s syndrome
D. Cri-du-chat syndrome
E. Cystic fibrosis
F. Kleinfelter's syndrome
G. Kallmann’s syndrome
H. Silver Russell syndrome
Instructions: From the given list of options, choose the single best answer for the given set of
questions. Each option maybe used once, more than once or not used at all.
1. A young mother brings her child to the paediatric OPD as she noticed he has absence of skin
on his scalp area. O/E- micrognathia, microcephaly, umbilical hernia and a pansystolic murmur of
VSD are present.

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2. A 5-year-old child is brought to the hospital with complaints of repeated chest infections and
GI problems since birth. His sweat chloride levels are diagnostic.
3. A tall adult male, with absence of secondary sexual characters, anosmia, cleft lip and palate
and mostly normal mental development.
4. A young female, with delayed puberty, cubitus valgus, heart defects and mild mental
retardation.
5. A mother brings her 2 year old with respiratory infection to the OPD. O/E he has simian
crease, retarded physically and mentally, large tongue, and a VSD.
5-Options
A. Duodenal atresia
B. Jejunal atresia
C. Oesophageal atresia
D. Necrotising enterocolitis
E. Malrotation
F. Malrotation with volvulus
G. Hirschprung's disease
H. 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, blood
in the stools and increasing ventilatory requirements
2. A baby girl born at 36 weeks is transferred to NICU on day 1 with cyanosis and respiratory
distress after attempted feeding
3. A baby boy born at term is transferred to NICU on day 2 with abdominal distension and failure
to pass meconium.
4. A baby boy born at term is transferred to NICU on day 10 with sudden collapse. He has bilious
aspirates, some blood in the stool and a silent abdomen.
5. A baby boy born at 32 weeks is transferred to NICU on day 1 with bilious vomitting. On
examination, he has features of Trisomy 21.
6-Options
A. Atrial septal defect
B. Ventricular septal defect
C. Patent ductus arteriosus
D. Coarctation of aorta
E. Ebstein anomaly
Instructions: Match the correct physical sign/presenting symptoms with the lesion of congenital heart
disease:1. A 17 year old presents with headache, leg fatigue and claudication. On examination there is
an absence of femoral pulses with a mid-systolic murmur heard over the back.
2. A 18 year old presents for a health checkup with no symptoms. On examination there is a
widely split fixed 2nd heart sound.
3. An 18 year old presents with palpitations. On auscultation there is a quintuple cardiac
cadence.
4. A 7 year old presents with breathlessness. On auscultation there is a continuous murmur.
5. A 3 year old presents with breathlessness and pedal oedema. On auscultation there is a sharp
holosystolic murmur heard along the left sternal border.
7-An 18-year-old man was admitted to casualty at 3 am having collapsed at a nightclub. A
friend, 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

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6 x 109/L White cell count 25. 2. What is the diagnosis? Options : Choose 1 A. Hypertrophic pyloric stenosis G. Strangulated inguinal hernia F. On percussion of the liver there is no dullness. 3. Autoimmune haemolysis B.8 x 109/L Eosinophils 0. Appendicitis E. Investigations revealed: Haemoglobin 8.8 x 109/L Neutrophils 22. Meckel's diverticulum D. Thrombotic thrombocytopenic purpura 8-Options A.PEDIATRICS EXAMINATION REVIEW and that he usually did not drink much alcohol or take any drugs.3 g/L D-Dimer screen 3. and an abdominal mass. Autoimmune thrombocytopaenia C. For the past 2 weeks he has had a cold. A 13 year old girl presents with a complaint of abdominal pain that she has experienced for 208 . more than once or not at all. headache and nausea. toxic granulation of neutrophils and platelet anisocytosis. and on palpation the liver is non-tender. a sore throat. A 2 year old girl is referred urgently to clinic having presented to her general practitioner with haematuria on two occassions.1 x 109/L Platelets 32 x 109/L Reticulocyte count 12% Prothrombin time 32 s Activated partial thromboplastin time 90 s Fibrinogen 0. Nephroblastoma I. is not tolerating fluids. Today he has vomited twice.0 mg/L Serum sodium 140 mmol/L Serum potassium 6. 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.5 x 109/L Lymphocytes 2. A 4 year old boy is brought into hospital by his mother.5 mmol/L Serum creatinine 200 mmol/L Lactate dehydrogenase 540 IU/L The blood film showed red cell fragmentation with polychromasia. Disseminated intravascular coagulation D. There is a firm mass with a poorly defined smooth lower border in the left upper quadrant of her abdomen. Intussusception B. Mesenteric adenitis C.4 x 109/L Basophils 0. Drug-induced haemolysis E. Each option may be used once.0 x 109/L Monocytes 0. and complains of central abdominal pain. She is now asymptomatic. Constipation H.3 mmol/L Serum urea 17.

The mother also informed the GP that despite losing weight her daughter was spending long time every day undertaking heavy exercises.8 x 109/L Eosinophils 0. When you ask to see his nappy. History reveals a bright red loss of blood per rectum with defaecation. Which of the following supports a diagnosis of anorexia nervosa in this patient:Options: Choose 5 A. poor appetite and chronic watery. Increased levels of growth hormone D. Cytomegalovirus infection D. His pulse was 104 beats per minute (bpm) regular. Spinal X-ray and HLA profile 209 . Binging on fatty food C. Loss of over 15% of body weight B. Increased levels of cortisol E.PEDIATRICS EXAMINATION REVIEW the past two weeks. 9-A 18-years-old adolescent had a bone marrow transplant from his sister for treatment of acute myeloid leukemia. Absence of depressive features F. penicillin V. A per rectum examination is too tender to perform and an anal fissure is noted at 6 o'clock. Use of diuretics 11-Options A. The GP reported that she has lost about 2 stones over the past 2 months and that although she was preoccupied by preparing food. green diarrhoea. Acute graft versus host disease B. you note a redcurrant jelly stool. co-trimoxazole and aciclovir. which she notes on the toilet paper. His abdomen was soft apart from mild tenderness in the right upper quadrant. Clostridium difficile infection C. a diffuse macular rash was visible. His medications included cyclosporin. Investigations revealed: Haemoglobin 12. 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.1 x 109/L Platelets 147 x 109/L Serum albumin 36 g/L Serum total bilirubin 64 µmol/L Serum alanine transferase 90 IU/L Serum alkaline phosphatase 135 IU/L Serum gamma glutamyl transferase 40 IU/L What is the diagnosis? Options: Choose 1 A. general malaise. Her abdomen is soft on palpation and on auscultation there are normal bowel sounds.05 x 109/L Basophils 0. No organs were palpable. mainly affecting the palms of his hands and the soles of his feet. Six weeks later he presented with a rash. Blood pressure 112/74 mmHg. 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. Side-effect of immunosuppression E.7 x 109/L Lymphocytes 1. which has worsened acutely today. On examination.4 g/dL Mean corpuscular volume (MCV) 90 fL White cell count 3.0 x 109/L Monocytes 0. Heart sounds were unremarkable and his chest was clear. Neurological examination was normal. she did not seem to be eating well. Bowel sounds were active. with a trace of blood on his anus. Amenorrhea G. 4. Her mother reported that she has been hording food in her bedroom.65 x 109/L Neutrophils 1.

more than once or not at all.PEDIATRICS EXAMINATION REVIEW B. He has some bluish white spots on his buccal mucosa. anorexia. conjunctivitis. Measles G. A 45-year-old man presents with swollen. A 6-month-old boy presents with a generalised rash after a bout of fever. 3. 2. A non-immunised child of 5 years presents with catarrh. A 45-year-old woman presents with wrist pain. Anti dsDNA and complement E. Joint aspiration and microscopy I. fever. Clinical history alone. Joint aspiration. He also has a 4/6 murmur. and splenomegaly. 12-Options A. Pelvic spring test G. Impetigo L. He also has a rash which does not blanch on pressure. 1. A 5-year-old Asian refugee child develops high fevers. 4. There is a small open wound. 5. which is worse at night and relieved by dangling the hand over the edge of the bed and shaking it. 4. Scabies C. A 45-year-old man who was on a fast developed acute pain in his toe. lymphadenopathy. Psoriasis E. erythema of the oral cavity. and palms. 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. 5. Bone scan C. congested conjunctivae. joint X-ray L. Meningitis F. joint X-ray J. Rheumatoid factor. Thomas test F. 210 . fever. Neurophysiology Instructions: For each of the patients described below choose the single most likely option from the above list of options. Roseola infantum K. A mother rings A&E saying that her 3-year-old boy has fever for 1 day and is drowsy. painful and stiff hands and feet and the symptoms are worse in the mornings. ECHO M. Varicella B. Sigmoidoscopy. lips. malaise. palatal petechiae. joint pains and a rash. Each option may be used once. swab and blood cultures K. 2. MRI D. 1. Ebstein-Barr virus J. A 3-year-old boy presents with oedema. biopsy and enema H. Kawasaki’s disease H. The wound is treated but 2 days later he develops pain in his knee. Herpes simplex virus I. more than once or not at all. A man has a bike accident and injures his leg. 3. Pityriasis rosea D. A 17-year-old African male presents with sore throat. It does not respond to antibiotics. organomegaly.

nausea. A veterinarian presents with fever. A man who is on steroids for rheumatoid arthritis for a long period of time now presents with white patches in his mouth. Streptococcus C. Brucellosis E. depression. Tuberculosis F. 4. Abnormal organisation of cells in tissue 14-Options : A. anorexia.PEDIATRICS EXAMINATION REVIEW 13-Options : A. arthritis. Destruction of normally formed tissue 5. Teratogen F. Dystocia Instructions: Match the above options with the best descriptive statement from the choices given below:1. Candidiasis H. She also has abdominal pain. 2. His mother gives a history of tonsillitis. Poliomyelitis G. Staphylococcus D. Infectious mononucleosis I. Deformation B. 5. Disruption C. Dysplasia E. A 15-year-old patient presents with a rash and suboccipital lymphadenopathy. He was not immunised as a child. vomiting. A 35-year-old lady presents with myalgia. Chemical agent with a malign influence on development 2. Hepatitis N. Malformation D. 211 . Diphtheria K. Intrinsic defects in the pattern of development 4. Abnormal development of a structure caused by extrinsic forces 3. Coryza L. more than once or not at all. Her bilirubin is raised. sacro-ileitis and rash. hepatosplenomegaly. Herpes virus B. A 4-year-old boy presents to A&E with shock. 3. 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. Rubella J. malaise. He is found to have myocarditis. and fever.) 1. Meningitis M.

PEDIATRICS EXAMINATION REVIEW Answer &Teaching Notes for Questions (PART 3) 212 .

Avascular necrosis D. Bamboo spine 3. Haemophilus influenza epiglottitis C. Most important biochemical abnormality is elevated alkaline phosphatase and normal calcium levels. 1. 2. Narrowing of joint space. Osteoarthritis F. In late stages X-ray shows bamboo spine where vertebrae are fused. 2-Options : A. It is usually monoostotic (affects one bone). Rheumatoid arthritis E.PEDIATRICS EXAMINATION REVIEW 1-Options : A. Paget’s disease Instructions: Choose the most appropriate disease for the radiological description. Ankylosing spondylitis C. Ankylosing spondylitis affects the spine in young adults and causes restricted spinal mobility. Joint space narrowing. 4. marginal osteophytes and subchondral cysts. Pneumococcal infection 213 . Paget’s disease is due to a disorder of bone remodelling. Mixed osteoblastic and osteolytic lesions in the left tibia. Teaching Notes for Question 1 Theme: Radiographic abnormalities in rheumatological disease Rheumatoid arthritis and osteoarthritis features are as what given in the question. Streptococcal throat infection B. There is also cortical thickening. Osteomalacia B. juxtaarticular osteoporosis and bony erosions.

A 16 year old boy presents with fever. . He has enlarged cervical lymph nodes. Erythema infectiosum I. Secondary bacterial infection is an important complication of measles infection particularly in malnourished children. Mortality is 10% and 15% of patients who recover will have residual neurological deficit. A maculopapular rash typically first appears behind the ears and spreads to involve the face and then the trunk and limbs. A 8 year old boy presents with malaise. They are seen early in the illness.subacute sclerosing panencephalitis (SSPE) is a rare complication caused by latent measles virus infection which on reactivation 5-10 yrs later results in a degenerative CNS disorder. Kawasaki’s disease E. Peripheral blood count reveals lymphocytosis.PEDIATRICS EXAMINATION REVIEW D. This severe complication usually manifests 7-14 days after initial presentation with drowsiness. Complications are due to infection by the measles virus itself -respiratory.gov. seizures. 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. It is caused by parvovirus B19 a small single-strand DNA virus.laryngotracheobronchitis. Q2. often followed by an erythematous maculopapular rash on the trunk and legs. malaise. Q1. headache. Rubella L. palatal petechiae. 3. sore throat. Mumps J. On investigation there is lymphocytosis with atypical lymphocytes. Tuberculosis G. and the smear will show atypical lymphocytes. It presents usually in young adults with fever.typically asymmetrical and transient -aplastic crises . lymphadenopathy. Typhus F. Complications include -arthritis. a bright red rash appears on the cheeks. hepatitis and haemolytic anaemia. 4. On day 5-7 after symptom onset. more than once or not at all. splenomegaly. malaise.doh.this number increases to 80% of patients if there has been previous treatment with amoxicillin or ampicillin. Teaching Notes for Question 2 Theme: Diagnosis of childhood illness. These are CD8+ Tlymphocytes which are produced in response to EBV infected B-cells. fever and coryza 12 days after exposure to a sick sibling.uk/mmr/index.html. sore throat. 5. This is a case of infectious mononucleosis caused by Epstein Barr virus .seen in children with conditions which decrease red cell survival such as sickle cell 214 .a virus of the herpes group which infects B-lymphocytes. nausea. Each option may be used once. Q3. malaise. Resolution of the rash reveals a lacy reticular pattern on the limbs. Infectious mononucleosis H. Common symptoms include a mild prodromal illness consisting of fever. This is a case of erythema infectiosum also known as slapped cheek syndrome. A 6 year old girl presents with fever and a slapped cheek erythematous eruption on her cheeks. Koplik spots are pathognomonic 'grain of salt like' spots on the buccal mucosa located adjacent to the posterior molar. A mild erythematous maculopapular rash occurs in 3-15% of patients . anorexia. myalgia.postinfectious encephalomyelitis. A 6 year old boy presents with fever. cervical lymphadenopathy and erythema of palms and soles. conjunctivitis and fever. Varicella zoster K. bronchitis. 1. anorexia. and has painful swelling of both parotid glands. and rhinorrhea.ear . This is a case of measles caused by an RNA paramyxovirus spread by droplets. Incubation period is 8 to 14 days. giant celll pneumonia . focal neurological signs and coma. Measles infection presents with catarrh. Measles Instructions: For each of the patients described below choose the single most likely option from the above list. A 5 year old girl presents with conjunctivitis. On examination she has blue white punctate lesions on her buccal mucosa.otitis media -CNS . fever. 2.

more than once or not at all. 1. Thyroid function tests G. plasma concentration monitoring is advised on starting the treatment. (A) Blood dyscrasia with low WBC and frequent infections is a severe side effect. Q4. tremor. This is a case of Kawasaki disease Kawasaki disease is a vasculitis of unknown aetiology which generally occurs in those under 9 yrs of age. pancreatitis and myocarditis. Complications are orchitis. Q2. it should be given within 10 days of the start of the illness.2 °C 4.PEDIATRICS EXAMINATION REVIEW anaemia. polymorphous rash and cervical lymphadenopathy. arthritis. Patient on Sodium Valproate. erythema of the palms and soles (which desquamate in the second or third week of illness). jaundice. myocardititis and coronary artery aneurysm. Cardiovascular complications occur in the subacute phase of illness (+/_ day 10 to day 30). EEG D. (G) Liver failure is more common in children < 3 years old . Incubation period is 14 . drowsiness 3. Each answer may be used once. The patient is infective 7 days before and 9 days after parotid swelling starts. but may occur in any age. This is a case of mumps caused by a single stranded RNA paramyxovirus. Drug serum levels K.21 days. bilateral non-exudative conjunctivitis. strawberry tongue). INR F. with diplopia. with sore troat. Thrombocytosis is a late finding. Patient on carbamazepine feeling unwell. Male patient on phenothiazines comes with discharge from his breast Teaching Notes for Question 3 Theme: Drug(s) Q1. 3-Options : A. anorexia. Marrow biopsy C. thalassaemia major. 37. Liver biopsy I. with vomiting. fever and painful swelling of the parotids. These include pericarditis. oral changes-(swollen red lips. give the most appropriate investigation for each scenario. other haemolytic anaemias. meningitis. There should be no delay in giving IVIG once the diagnosis (which is a clinical one) is made. The pyrexial phase of the illness lasts up to 10 days and is usually accompanied by intense irritability and fractiousness. Patient on phenytoin. Q4. Breast ultrasound Instructions: Drug side effects-investigations. CT scan E. Q5. The infection presents with malaise. Liver function tests H. LFT should be monitored in first 6 months of therapy Q3. Prolactine levels J. Mortality is around 1% for Kawasaki disease due to acquired cardiovascular disease. Diagnostic criteria include fever. (J) Phenytoin levels vary in individuals. and low mood 2. (I)Neuroleptics by blocking dopamine D2 receptors may give extrapyramidal effects and 215 . Full blood count B. and temp.

anosmia. Patau’s syndrome is trisomy 13. A young female. Intussusception Instructions: Please match the most likely diagnosis with the scenario described below 1. Malrotation with volvulus G. heart defects and mild mental retardation. some blood in the stool and a silent abdomen. 3. 4. Cri-du-chat syndrome E. 2. Down's syndrome B. Down’s syndrome is trisomy 21. Oesophageal atresia D.PEDIATRICS EXAMINATION REVIEW hyperprolactinemia. Kallmann’s syndrome H. Patau’s syndrome D. A baby boy born at term is transferred to NICU on day 2 with abdominal distension and failure to pass meconium.micrognathia. 4-Options A. Necrotising enterocolitis E. large tongue. Kallman’s syndrome is characterised with the features given in the question. Malrotation F. retarded physically and mentally. 5. more than once or not used at all. blood in the stools and increasing ventilatory requirements 2. O/E he has simian crease. He has bilious aspirates. Kleinfelter's syndrome G. Duodenal atresia B. A 5-year-old child is brought to the hospital with complaints of repeated chest infections and GI problems since birth. A baby girl born at 36 weeks is transferred to NICU on day 1 with cyanosis and respiratory distress after attempted feeding 3. 2. A young mother brings her child to the paediatric OPD as she noticed he has absence of skin on his scalp area. Turner's syndrome C. A baby boy born at term is transferred to NICU on day 10 with sudden collapse. Cystic fibrosis F. A mother brings her 2 year old with respiratory infection to the OPD. O/E. 4. cleft lip and palate and mostly normal mental development. choose the single best answer for the given set of questions. 216 . A baby girl born at 26 weeks is transferred to NICU on day 3 with abdominal distention. 5. Teaching Notes for Question 4 Theme: Chromosomal disorders 1. 5-Options A. with delayed puberty. His sweat chloride levels are diagnostic. Silver Russell syndrome Instructions: From the given list of options. Jejunal atresia C. and a VSD. microcephaly. A tall adult male. Cystic fibrosis occurs due to abnormality in long arm of chromosome 7. 4. 1. umbilical hernia and a pansystolic murmur of VSD are present. 3. cubitus valgus. Hirschprung's disease H. Turner’s syndrome is 45 XO karyotype. with absence of secondary sexual characters. Each option maybe used once.

6-Options A. abdominal distension. there is a higher incidence in males (4:1) with an equal sex incidence in 'long segment Hirschsprung's' disease. Teaching Notes for Question 5 Theme: Neonatal intestinal obstruction 1. On examination. The disorder shows a familial tendency. There is usually an associated tracheo-oesophageal fistula (90%). A 17 year old presents with headache. Patent ductus arteriosus D.'short segment Hirschsprung's disease'.000 live births. 5. Aganglionosis may be restricted to the rectum or rectosigmoid region . Causes of bowel obstruction in the neonate are: -atresia of any part of the gastrointestinal tract -duodenal. A 18 year old presents for a health checkup with no symptoms. he has features of Trisomy 21. This is followed by a definitive pull through procedure at 3-12 months of age. coughing with episodes of cyanosis exacerbated by feeding. Duodenal atresia occurs in 1 in 10. There is a high incidence of aspiration pneumonia. Babies present with vomiting. Babies present with bilious or non-bile stained vomiting.'long segment Hirschsprung's disease'. Clinical symptoms are usually present within the first few days of life with delayed passage of meconium. X-ray shows the typical 'double-bubble'. Children with 'short segment Hirschsprung's disease' can present later in life. Coarctation of aorta E. During embryological development the intestines herniate into the umbilical cord at 4. On examination there is a 217 .Treatment is surgicalduodenoduodenostomy. There is an association with Down's syndrome (30%) as well as cardiac anomalies. choking. jejunoileal atresia. A second enema 24 hrs after the first shows retention of contrast and may reveal the transition zone between normal bowel and the distal aganglionic intestine. 3. Rectal biopsy confirms the diagnosis. rectal passage of blood and peritonitis. 2. Barium enema shows a narrowed distal colon with proximal dilation. leg fatigue and claudication. large bowel atresia -mechanical obstruction -meconium ileus. including pyloric stenosis. and in cases of volvulus with abdominal distension. Atrial septal defect B. malrotation and biliary atresia.Hirschsprung's disease -intussusception may occasionally occur in the neonate Later in the neonatal period other conditions should be considered. Enterocolitis is a severe complication and presents with diarrhoea. They then return to the abdomen and rotate in an anticlockwise direction. and there is an association with Down's syndrome (5-15%) and cardiac malformation (2-5%).10 weeks gestational age. There is often polyhydramnios in pregnancy.The site of obstruction is most commonly in 2nd part of duodenum with hypertrophy of the proximal duodenum. bilious vomiting. In 'short segment Hirschsprung's disease'. poor feeding and failure to thrive. gross abdominal distension and circulatory collapse. Treatment usually involves an initial colostomy proximal to the aganglionic bowel. NEC is a disease of prematurity and impaired gut perfusion is thought to be responsible for the clinical picture.PEDIATRICS EXAMINATION REVIEW 5. 2. The child presents with frothing from the mouth. 4. On examination there is an absence of femoral pulses with a mid-systolic murmur heard over the back. Failure of this normal process results in malrotation which results in a narrow mesenteric stalk which predisposes to midgut volvulus and intestinal obstruction. Hirschprung's disease is caused by the absence of ganglion cells in the myenteric plexuses of the distal bowel. Ventricular septal defect C. or may extend above the sigmoid . A baby boy born at 32 weeks is transferred to NICU on day 1 with bilious vomitting. meconium plug -volvulus -malrotation -imperforate anus -failure of normal peristalsis . Oesophageal atresia has an incidence of about 1 in 2500 live births. Ebstein anomaly Instructions: Match the correct physical sign/presenting symptoms with the lesion of congenital heart disease:1. with a history of chronic constipation since birth and poor weight gain.

The upper extremities and thorax may be more developed than the lower extremities. 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. On auscultation there is a quintuple cardiac cadence. 7-An 18-year-old man was admitted to casualty at 3 am having collapsed at a nightclub. Investigations revealed: Haemoglobin 8. A friend. Q3.1 x 109/L Platelets 32 x 109/L Reticulocyte count 12% Prothrombin time 32 s Activated partial thromboplastin time 90 s Fibrinogen 0. Surgical ligation of the ductus gives good results. Enlarged and pulsatile collateral vessels may be palpated in the intercostal spaces anteriorly. discrete coarctation are asymptomatic. A 7 year old presents with breathlessness. In this condition. The S1 is split. in the axillae. during inspiration.5 x 109/L Lymphocytes 2. Attention is usually directed to the cardiovascular system when a heart murmur or hypertension in the upper extremities and absence.0 x 109/L Monocytes 0. or posteriorly in the interscapular area. 5. 3. Q5. Prognosis depends on the size of shunt . systolic ejection murmur at pulmonary area are some of the examination findings.PEDIATRICS EXAMINATION REVIEW widely split fixed 2nd heart sound. widely split and fixed S2. Right ventricular lift. An 18 year old presents with palpitations. Hence a quintuple cadence is heard on auscultation. Usually. Q2. Features of left ventricular hypertrophy are found on examination and ECG.4 x 109/L Basophils 0. Infective endocarditis is more common. The extremities can feel cold and claudication with exercise may occur due to diminished blood flow from the aorta to the lower extremities. Large shunts cause high mortality from cardiac failure. On auscultation there is a sharp holosystolic murmur heard along the left sternal border. the atria are linked via the defect.a portion of the right ventricle is atrialized.8 x 109/L Neutrophils 22. who had accompanied him in the ambulance but subsequently left A and E before speaking to a doctor. A midsystolic murmur over the anterior part of the chest. A 3 year old presents with breathlessness and pedal oedema. This is a case of coarctation of the aorta.8 x 109/L Eosinophils 0. 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. marked diminution. back. 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.Ebstein anomaly (congenitally malpositioned tricuspid valve) is a cardiac defect in which the leaflets of the tricuspid valve are displaced into the right ventricle . 4.large defects cause disability by the age of 40. 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. or delayed pulsations in the femoral arteries are detected on physical examination. ECG shows right axis deviation and features of right ventricular hypertrophy. Q4. Most children and young adults with isolated. On auscultation there is a continuous murmur. 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.6 x 109/L White cell count 25.a fixed split S2. there is an increase in venous return to the right ventricle which delays closure of the pulmonary valve.3 g/L 218 . In an ASD. pressure in the aorta is higher than the pressure in the pulmonary artery throughout systole and diastole thus a continuous murmur is heard. Teaching Notes for Question 6 Theme: Congenital heart diseases Q1. In ventricular septal defect the left ventricular pressure is higher than that of the right ventricle during systole resulting in the characteristic holosystolic murmer. therefore inspiration produces no net pressure change between themthe timing of the pulmonic valve closure does not change with regards to aortic valve closure and has no effect on the splitting of S2 . Headache and epistaxis are symptoms. Atrial septal defect is usually asymptomatic until middle age. Patent ductus arteriosus is usually asymptomatic but can present with left ventricular failure or pulmonary hypertension.

Autoimmune thrombocytopaenia C. Each option may be used once. 1. and complains of central abdominal pain. When you ask to see his 219 . Drug-induced haemolysis E. Disseminated intravascular coagulation D. which has worsened acutely today. A 13 year old girl presents with a complaint of abdominal pain that she has experienced for the past two weeks. Autoimmune haemolysis B. Mesenteric adenitis C. which she notes on the toilet paper. Meckel's diverticulum D. A 4 year old boy is brought into hospital by his mother. 4.3 mmol/L 17. 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.5 mmol/L 200 mmol/L 540 IU/L The blood film showed red cell fragmentation with polychromasia. 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. headache and nausea. Hypertrophic pyloric stenosis G. History reveals a bright red loss of blood per rectum with defaecation. low fibrinogen.4methylenedioxymethamphetamine (MDMA).PEDIATRICS D-Dimer screen Serum sodium Serum potassium Serum urea Serum creatinine Lactate dehydrogenase EXAMINATION REVIEW 3. high neutrophil count and thrombocytopenia are all indicative of disseminated intravascular coagulation (DIC). 3. and an abdominal mass. What is the diagnosis? Options : Choose 1 A. anaemia. Strangulated inguinal hernia F. There is a firm mass with a poorly defined smooth lower border in the left upper quadrant of her abdomen. Thrombotic thrombocytopenic purpura Teaching Notes for Question 7 Theme: DIC and Ecstasy The coagulopathy. A 2 year old girl is referred urgently to clinic having presented to her general practitioner with haematuria on two occassions. Intussusception B. She is now asymptomatic. is not tolerating fluids. 8-Options A. The causative agent is likely to be ecstasy/3. A per rectum examination is too tender to perform and an anal fissure is noted at 6 o'clock. Today he has vomited twice. raised D-dimers. Nephroblastoma I. Her abdomen is soft on palpation and on auscultation there are normal bowel sounds. and on palpation the liver is non-tender. more than once or not at all. Constipation H. 2.0 mg/L 140 mmol/L 6. For the past 2 weeks he has had a cold. a sore throat. On percussion of the liver there is no dullness. Appendicitis E. In addition the renal failure with high potassium levels (K) and lactate dehydrogenase (LDH) are suggestive of associated rhabdomyolysis. toxic granulation of neutrophils and platelet anisocytosis.

Side-effect of immunosuppression E. mainly affecting the palms of his hands and the soles of his feet. 9-A 18-years-old adolescent had a bone marrow transplant from his sister for treatment of acute myeloid leukemia. Acute graft versus host disease B. you note a redcurrant jelly stool. This will prevent a diagnosis of appendicitis being missed. Enlarged cervical lymph nodes on examination with a history of a previous URTI points towards mesenteric adenitis. Teaching Notes for Question 8 Theme: Abdominal conditions in children 1. Clostridium difficile infection C. 220 . Cytomegalovirus infection D.8 x 109/L 0. Its clinical presentation mimics that of acute appendicitis. hepatitis (tender right upper quadrant (RUQ) and raised alanine transaminae (ALT) and bilirubin) and gastro-enteritis (diarrhoea – usually described as green and watery). but an association with streptococcal infections of the upper respiratory tract has been reported. where the location of tenderness tends to be fixed.1 x 109/L 147 x 109/L 36 g/L 64 µmol/L 90 IU/L 135 IU/L 40 IU/L What is the diagnosis? Options: Choose 1 A. The child with appendicitis is usually quiet with facial flushing. co-trimoxazole and aciclovir.0 x 109/L 0. His pulse was 104 beats per minute (bpm) regular. On examination. general malaise. Bowel sounds were active. No organs were palpable. 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).05 x 109/L 0. a diffuse macular rash was visible.4 g/dL 90 fL 3. Six weeks later he presented with a rash. Heart sounds were unremarkable and his chest was clear. penicillin V.65 x 109/L 1. there should be careful discussion with the parents to observe for worsening symptoms despite analgesics and fluids. poor appetite and chronic watery. If the child is not admitted for observation. with a trace of blood on his anus. Pyrexia usually resolves rapidly with mesenteric adenitis. His abdomen was soft apart from mild tenderness in the right upper quadrant. On examination. Mesenteric adenitis is caused by inflammation of the mesenteric lymph nodes. as opposed to appendicitis. 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 12. The longer history of this case suggests mesenteric adenitis as the single most likely diagnosis. His medications included cyclosporin.7 x 109/L 1. the site of abdominal tenderness may shift with a change in patient position.PEDIATRICS EXAMINATION REVIEW nappy. Unfortunately (for diagnostic purposes) appendicitis has few signs of peritonitis in the early stages. Neurological examination was normal. It is most often associated with a viral infection. Blood pressure 112/74 mmHg. green diarrhoea.

It may also affect males who make less than 10% of all patients. The onset is usually in the teen years. Increased levels of growth hormone D. Binging on fatty food C. Prognosis is variable. Which of the following supports a diagnosis of anorexia nervosa in this patient:Options: Choose 5 A. Rheumatoid factor. MRI D. Use of diuretics Teaching Notes for Question 10 Theme: Anorexia Anorexia nervosa is a serious psychiatric disorder affecting girls and young women. patients may also have elevated growth hormone and cortisol levels. joint X-ray J. swab and blood cultures K. Absence of depressive features F. Patients usually present with 15% or more body weight loss. Anti dsDNA and complement E. Amenorrhea G. Joint aspiration and microscopy I. The GP reported that she has lost about 2 stones over the past 2 months and that although she was preoccupied by preparing food. Sigmoidoscopy. Patients routinely avoid fattening food and indulge in excessive exercises. Bone scan C. Her mother reported that she has been hording food in her bedroom. It is very rare in middle age and beyond. Clinical history alone. In addition to amenorrhoea. biopsy and enema H. 11-Options A. Treatment of the condition may include hospital admission to restore weight and psychotherapy. joint X-ray 221 . The main psychopathology behind the illness is distortion of body image and morbid fear of fatness.PEDIATRICS EXAMINATION REVIEW 10-A 14 year old girl was referred to the local child psychiatrist by her GP who was concerned about her weight loss. Spinal X-ray and HLA profile B. Increased levels of cortisol E. Loss of over 15% of body weight B. Pelvic spring test G. The mother also informed the GP that despite losing weight her daughter was spending long time every day undertaking heavy exercises. Thomas test F. self-induced vomiting and use of diuretics. she did not seem to be eating well. Joint aspiration.

This is rheumatic fever. organomegaly. 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. Diagnosis depends upon finding urate crystals in tissues and synovial fluid. A 45-year-old man presents with swollen. Meningitis F. Ebstein-Barr virus J. 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. 5. 2. Rheumatoid factor and joint X-ray is needed here as this is a case of rheumatoid arthritis which presents with swollen. congested conjunctivae. Neurophysiology 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. Impetigo L. 3. 12-Options A. 2. anorexia. 4. Teaching Notes for Question 11 Theme: Differential diagnosis of joint pains 1. 3. more than once or not at all. infection and diuretics. A man has a bike accident and injures his leg. joint pains and a rash. surgery. A 5-year-old Asian refugee child develops high fevers. He has some bluish white spots on his buccal mucosa. Kawasaki’s disease H. fever. A 17-year-old African male presents with sore throat. which is worse at night and relieved by dangling the hand over the edge of the bed and shaking it. A 45-year-old woman presents with wrist pain. Joint aspiration. palatal petechiae. malaise. conjunctivitis. 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. A 6-month-old boy presents with a generalised rash after a bout of fever. ECHO M. He should have a full cardiac assessment. A non-immunised child of 5 years presents with catarrh.PEDIATRICS EXAMINATION REVIEW L. painful and stiff hands and feet and the symptoms are worse in the mornings. 5. lips. There is a small open wound. Joint aspiration and microscopy is needed here as this is a case of gout which is due to deposition of sodium monourate crystals in the joints and may be precipitated by trauma. Varicella B. Psoriasis E. 4. Measles G. 2. and palms. The wound is treated but 2 days later he develops pain in his knee. 3. 222 . erythema of the oral cavity. Herpes simplex virus I. painful and stiff hands and feet and the symptoms are worse in the mornings. 1. 5. lymphadenopathy. Scabies C. fever. starvation. It does not respond to antibiotics. A mother rings A&E saying that her 3-year-old boy has fever for 1 day and is drowsy. more than once or not at all. He also has a 4/6 murmur. Roseola infantum K. Pityriasis rosea D. and splenomegaly. He also has a rash which does not blanch on pressure. 1. A 45-year-old man who was on a fast developed acute pain in his toe. A 3-year-old boy presents with oedema. 4.

Koplik spots are pathognomonic grain of salt like spots on the buccal mucosa. congested conjunctivae. Although HHV-6A has not yet been associated with disease. Malformation D. Abnormal organisation of cells in tissue Teaching Notes for Question 13 Theme: Dysmorphogenesis Types of problems in morphogenesis include: a. It presents with sore throat. Breakdown of normal tissue. Disruption C. erythema of the oral cavity. Destruction of normally formed tissue 5. HHV-6B is also a major cause of febrile seizures without rash during infancy. and desquamation of the skin of the fingertips. and changes in the skin and mucous membranes such as oedema. In older age groups. It presents with catarrh. conjunctivitis.disruption. lymphadenopathy. HHV-6B can cause exanthem subitum (roseola infantum). Q4: This is a case of infection caused by Epstein Barr virus which preferentially infects B lymphocytes. abnormal ear shape and size in many syndromes 14-Options : A. Abnormal organisation of cells in tissue. 13-Options : A.g. lips. Teratogen F. Dystocia Instructions: Match the above options with the best descriptive statement from the choices given below:1. Streptococcus C. palatal petechiae. febrile. It is an acute. incubation period is 721 days. It is characterised by unresponsiveness to antibiotics. amniotic bands d. fever.g. Q3: This is a case of meningitis as the child has a fever and has been drowsy. multi-system disease of children. breech deformation sequence c. Unusual forces acting on normal tissues. He also has the characteristic non-blanching rash of meningitis.malformation. focal encephalitis.g. e. a common illness characterised by fever with subsequent rash. Abnormal development of a structure caused by extrinsic forces 3. anorexia. malaise. Staphylococcus 223 . Dysplasia E.dysplasia e. HHV-6B has been associated with mononucleosis syndromes. and palms. Deformation B. Q5: This is a case of measles caused by RNA paramyxovirus spread by droplets. defective formation of tissue. Infection with HHV-6 frequently develops during infancy as maternal antibody wanes. Chemical agent with a malign influence on development 2. and (in immunocompromised hosts) pneumonitis and disseminated disease Q2: This is a case of Kawasaki disease (mucocutaneous lymph node syndrome.g. and splenomegaly.PEDIATRICS EXAMINATION REVIEW Teaching Notes for Question 12 Theme: Fever with rash Q1: This is a case of Roseola infantum. fever. Herpes virus B. Intrinsic defects in the pattern of development 4. congenital heart defects b.deformation e. nonsuppurative cervical adenitis. e. The organism can and must be isolated.

hepatosplenomegaly. The incubation period 14-21 days. toxaemia or conducting system involvement. A 15-year-old patient presents with a rash and suboccipital lymphadenopathy. anorexia. anorexia. sacro-ileitis and rash. Candidiasis H. Coryza L. 5. depression. 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.) 1. sacro-ileitis and rash. 4. Poliomyelitis G. 224 . Q4: This is a case of brucellosis which is common in the Middle East. tonsillitis and a false membrane over the fauces. Her bilirubin is raised. She also has abdominal pain. Diphtheriae usually presents with a brassy cough. Q2: This is a case of Rubella caused by a RNA virus. A 4-year-old boy presents to A&E with shock. He was not immunised as a child. malaise. arthritis. malaise. and fever. Diphtheria K. hepatosplenomegaly. His mother gives a history of tonsillitis. Shock may occur from myocarditis. 2. A veterinarian presents with fever. Small joint arthritis may also be present. Brucellosis is more commonly found amongst vets and farmers. vomiting. depression. nausea. Brucellosis E. A 35-year-old lady presents with myalgia. He is found to have myocarditis. Q5: This is a case of candidiasis which is commonly found in patients who have low immunity. The following signs and symptoms are present: fever. Infectious mononucleosis I. Hepatitis N. Teaching Notes for Question 14 Theme: Diagnosis of infections Q1: This is a case of diphtheria which is caused by corynebacterium diphtheriae. Tuberculosis F. This is evident from the fact that the patient has jaundice (yellow sclera) and pain in the region of his right upper abdomen. The toxin may cause polyneuritis. A diagnosis is made using blood cultures. 3. vomiting. Rubella J. It presents with a mild macular rash and suboccipital lymphadenopathy.PEDIATRICS EXAMINATION REVIEW D. A man who is on steroids for rheumatoid arthritis for a long period of time now presents with white patches in his mouth. more than once or not at all. Q3: This is a case of hepatitis. arthritis. Meningitis M.