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1-Theme:Vitamin Dependant States


Ascorbic Acid

















Vitamin E
Which of the above options best fit the statements below?
A deficiency leads to nyctalopia.
Vitamin E

Incorrect - The correct answer is Retinol

A deficiency leads to Pellagra.
Ascorbic Acid

Incorrect - The correct answer is Niacin

A deficiency results in dermatitis and seborrhoea.

Incorrect - The correct answer is Biotin

Retinal is another term for vitamin A. Deficiency results in ocular manifestations
and visual impairment, e.g. nyctalopia, photophobia, xerophthalmia and

keratoconjunctivits. Niacin is a constituent of coenzyme.essential for metabolism.
A deficiency results in Pellagra. Biotin is a constituent of co-enzymes involved in
CO2 transfer. .A deficient state results in dermatitis and seborrhea.

2-A 4-year-old girl is referred with behavioural problems. Her speech is
generally well-formed. However, she finds it difficult to sustain
conversations because she talks obsessively about her own interests. She
avoids eye contact. She plays on her own for hours with her dolls,
dressing and undressing them repeatedly. Attempts to play with others
results in tantrums. Born at 39+6/40, 3.18kg no neonatal problems. No
drugs, allergies. Fully immunised. No FH/SH of note.
On examination she is on the 50% for height and weight. There are no
specific abnormalities to find.
What is the most likely diagnosis?
(Please select 1 option)


Incorrect answer selected

Chronic secretory otitis media
Global developmental delay
Pervasive developmental disorder

This is the correct answer

Speech delay, isolated

This child has problems with reciprocal social interaction, with repetitive obsessive
play. Her speech is normal in form. These symptoms are characteristic of
Asperger syndrome. This is part of the spectrum of Pervasive Developmental
Disorders, and is regarded by some as 'high-functioning autism'. Such children
may be regarded as eccentric by their peers.

3-A 15-year-old boy plays competitive football. Following an accidental
clash of heads he initially seems to recover, but a few minutes later he has
a 5 minute generalised convulsion. This starts with eye-rolling, then
stiffness of all limbs, followed by rhythmic shaking and incontinence. He is
placed in the recovery position and an ambulance called. He is generally
fit and well. Full term normal delivery with no neonatal complications.
Immunisations up to date. There is no FH/SH of note.
On examination he is slightly drowsy but responds to voice. RR is 12/min
and HR 70/min. There are no abnormalities to find except bilaterally upgoing plantars. BM stix shows a glucose of 5.4 mmol/l.
What is the most likely diagnosis?
(Please select 1 option)

Drug ingestion
Extradural haematoma

Incorrect answer selected

Idiopathic epilepsy
Post-traumatic seizure

This is the correct answer

Subdural haematoma

The history suggests a brief generalised seizure following a minor head injury.
This is likely to have no long-term sequelae. A single episode cannot be
considered epilepsy. About 5% of children will have at least 1 seizure during their
lives, mostly related to fever. Fatigue, particularly with alcohol ingestion, is
another common trigger.

4-A 17-year-old woman presented six hours after taking 30 g of
Which of the following factors is most likely to predict an increased risk of
hepatotoxicity from the paracetamol?
(Please select 1 option)

Anorexia nervosa

This is the correct answer

Consumption of 20 units of alcohol since taking the paracetamol


answer selected
Gilbert's disease
Ingestion of amitriptyline with the paracetamol
Smoking 20 cigarettes per day

High risk groups in paracetamol overdose include:

malnourished patients (anorexia nervosa/bulimia

patients taking enzyme inducing drugs (e.g.
carbamazepine, phenytoin, rifampicin and St
John's wort)

patients with induced liver enzymes due to
chronic ethanol abuse

human immunodeficiency virus (HIV) positive

Traditional newborn screening methods involve screening of only these high-risk infants. neonatal jaundice. A previous dental extraction resulted in bleeding that had required two unit transfusion. which would include Type I. low birth weight. prematurity. 6-What proportion of the total number of children born with some degree of hearing impairment are detected by neonatal screening of babies considered at 'high-risk' of having congenital hearing loss? (Please select 1 option) 25% 50% This is the correct answer 75% 95% Incorrect answer selected 100% A number of risk factors are associated with neonatal hearing loss. rubella or . not factor VIII concentrate. although there is some controversy in Type II B as it is thought that DDAVP can exacerbate thrombocytopenia that can accompany this type of Von Willebrand's. The history tells us that she has mild disease.See review in Clinical Medicine Vol 3 No 2 2003. The risk factors considered include: family history of hearing loss. 5-A 17-year-old girl with mild Von Willebrand’s disease is scheduled for dental extraction. What is the most appropriate treatment prior to dental surgery? (Please select 1 option) Cryoprecipitate DDAVP This is the correct answer Fresh frozen plasma Incorrect answer selected High purity factor VIII concentrate Recombinant factor VIII concentrate DDAVP is the choice treatment for mild vonWillebrand disease. It is of no use in Type III – severe Von Villebrand's disease. You would not use cryoprecipitate or Fresh frozen plasma in these patients in this era due to potential viral transmission risk from blood products. and the majority of Type II. For severe disease you would use a Von Willebrand factor concentrate.

For each child detected by high-risk screening. craniofacial deformity. Universal newborn hearing screening is now being introduced to ensure most children have their hearing loss detected from birth. Immobilization of fractures is also well tolerated in general. Only half of the total number of children born with a hearing impairment is detected by these high-risk screening programs. is usually well tolerated. 8-A post-marketing surveillance study of a new heart failure therapy to the market was carried out on 10. if not excessive. anoxia. bacterial meningitis and Apgars score of 0-3. another child goes home undetected. The bone is more elastic. The other half has no obvious risk factors to alert parents or professionals to the presence of the hearing loss. Healing is usually prolonged Angulation and displacement of fractures are poorly tolerated. Correct Bone is generally more brittle. Fractures in children usually heal quicker than similar fractures in adults. The periosteum in children is thick and can often be used as an aid to hold reduction. Due to the remodelling potential of paediatric fractures angulation and displacement. Stiffness of adjacent joints is common following fracture immobilisation. 7-Relating to fractures in children:(Please select 1 option) The periosteum is thicker and more highly developed.000 subjects who had completed clinical trials. Which one of the following most accurately reflects the information generated from such a study? (Please select 1 option) Adverse events profile This is the correct answer Cost benefit analysis Cost effectiveness Comparative therapeutic efficacy Incorrect answer selected Drug potency Post-marketing surveillance/observational studies (phase IV studies) generally are designed to assess the potential side effects of new drugs but under everyday conditions and with a minimum of intervention.non-bacterial intrauterine infection. In .

PMS typically include patients from more extreme age groups.contrast to the randomized controlled trials. though 30% may have further febrile seizures. There is no FH/SH of note. antipyretics. Respiratory rate is 20/min and heart rate 120/min. On examination she alert though febrile at 39. PMS enroll a large number of patients. Immunisations up to date. Full term normal delivery with no neonatal complications. 10-Theme:Drugs During Pregnancy A Aminoglycosides B Aspirin C Beta Blockers D Lithium E Phenytoin . but became very warm this afternoon just prior to the episode. typically several thousands. simple Correct Meningitis The history is of a febrile child having a brief generalised convulsion with rapid recovery. complex Febrile convulsion. patients with comorbidity or other risk factors. They should be told how to control fever (strip down. Chest is clear and urinalysis negative. What is the most likely diagnosis? (Please select 1 option) Cerebral abscess Encephalitis Febrile convulsion. room at 19C. 9-A 17 month old girl presents with eye-rolling followed by generalised shaking of the limbs for 3 minutes. No previous history of note. with flushed cheeks. She has a crusty nasal discharge. and tepid sponge of head). She has had a cold for 3d. Parents should be advised that the prognosis is excellent. The picture is typical of a Simple Febrile Convulsion. The likely precipitant is a viral URTI. Comparative efficacy has already been undertaken in Phase III studies (RCTs) but can also be undertaken as part of specific RCT studies later in the drugs development and potency usually in phase I and II studies. In order to cover a wide spectrum of patients and to observe rare events with sufficiently high probability.8°C.

. Ebstein's anomaly being the most common (3% of all cases). If given in early pregnancy is associated with a wide variety of cardiac defects with approximately 8% having severe cardiac disease.F Pethidine G Sodium Valproate H Sulphonamides I Thiazide diuretics J Warfarin Select the drug from the above list of options that is most likely to be associated with the following effects if taken during pregnancy: This drug taken in early pregnancy may result in Ebstein's anomaly in 3% of cases. growth impairment and learning difficulties. Lithium Correct This drug may result in a neural tube defect in approximately 2% of cases. Warfarin Incorrect . when taken in pregnancy may result in a baby being born with cranio-facial abnormalities. a drug used in the treatment of bipolar affective disorder. It is also associated with abnormalities of the face and digits. compromising cranio-facial abnormalities and learning difficulties as well as growth impairment. Sodium Valproate Correct This drug. Sodium Valproate tends to induce a neural tube defect in approximately 2% of pregnancies and therefore should be avoided.The correct answer is Phenytoin Lithium. Phenytoin is well known for causing the foetal hydantoin syndrome. compromising the foetal Valproate syndrome.

and it usually presents on the face and legs. cases of haematogenous osteomyelitis due to Haemophilus influenzae have almost been eradicated. Pseudomonas infection is a common organism in haemodialysis patients and intravenous drug addicts. . A diagnosis of osteomyelitis is suspected. What is the likely infecting organism? (Please select 1 option) Clostridium difficile Haemophilus influenzae Pseudomonas Salmonella Staphylococcus aureus Correct The commonest infecting organism in acute osteomyelitis in children over the age of four years is Staphylococcus aureus.11-Features consistent with Henoch-Schönlein syndrome include True / False An onset at 3 years Incorrect answer selected Microscopic haematuria Proteinuria Correct Correct An urticarial rash over the face Incorrect answer selected A joint effusion with polymorphonuclear leucocytosis of the synovial fluid Correct Henoch-Schonlein purpura (HSP) peak incidence is at three years of age. 12-A 5-year-old boy presents to Accident and Emergency complaining of acute pain over his upper tibia. An urticarial rash can occur. With immunisation. He is febrile and he refuses to move his leg. though the typical non-thrombocytopenic purpura is much more common in HSP. Salmonella is the characteristic organism in sickle cell anaemia.

6) This is the correct answer This patient has symptoms suggestive of hypercalcaemia.9 pmol/L (9-22) Incorrect answer selected Prolactin of 1000 mU/L (<450) Serum alkaline phosphatase of 350 iu/L (50-110) Serum corrected calcium 2. 26% for neglect. . which are clinically significant. Free T is at the lower end of the normal range which is often the case in pregnancy and TSH is a better guide of thyroid function. It is also normal for serum alkaline phosphatase to rise by up to 4 times normal due to increased placental production. but which of these are clinically significant? (Please select 1 option) Detectable urinary human chorionic gonadotrophin Free thyroxine 8.  Parent indifferent. Macrosomic child Maternal smoking Correct Correct Incorrect answer selected Less than 18 months between the birth of children Correct Unfortunately.89 mmol/L (2. 37% were for physical injury. In 1993. intolerant or overanxious towards the child.13-A 17-year-old primigravida complains of constipation and arthralgia at 28 weeks gestation. 14-The following are risk factors for child abuse: True / False Infant separated from mother for more than 24 hours post-delivery. as is detectable urinary human chorionic gonadotrophin. 26% for sexual abuse.000 case conferences are held each year in England. child abuse is a common and important problem. 4 Hyperprolactinaemia is a normal finding in pregnancy. Correct Infant mentally or physically handicapped.2-2. Risk factors include:  Family history of violence. and 11% for emotional abuse. About 4050. A number of biochemical investigations are performed.

40% of 2nd and 3rd burns over the trunk and face.  Less than 18 months between the births of children. respiratory rate 25/min.  Mother less than 21 years old at the time of birth. No family or social history of note. Immunisations up to date.  History of mental illness.7°C. 15-A 3-year-old girl is brought in by ambulance from a house fire. Single or separated parent. Capillary refill time of 3 seconds. with blackening around the mouth and nostrils. He has extensive areas of peeling skin over the trunk and face.  Infant mentally or physically handicapped. as pulmonary oedema can be anticipated. 100% O2 should be given.  Infant separated from mother for more than 24 hours post-delivery.  Infant premature or low birth weight.  Socio-economic problems such as unemployment. no neonatal problems. On examination the temperature is 36. drug or alcohol addiction. pulse 130/min.  Step-parent or co-habitee present. The airway should be secured by immediate intubation before it becomes too oedematous and occludes completely. Full term normal delivery. Two large-bore cannulae . Shock can occur in the first few hours from loss of large amounts of plasma from denuded skin. What is the most important part of management? (Please select 1 option) Analgesia Incorrect answer selected Bag and mask ventilation Face mask oxygen Intubation This is the correct answer IV fluid bolus The picture is one of severe burns and smoke inhalation.  Parent abused or neglected as a child.

Maintenance and continuing losses should be calculated from the time of the fire. . and is decreased in splenectomies done for trauma. secondary hypersplenism. insulin infusion. pneumoniae. metabolic storage disease. 16-In diabetic ketoacidosis: True / False bicarbonate should be given if pH is less than 7. Correct Correct Is only a significant problem for two/three years post splenectomy. Is more common in children <5 years. Steroids may worsen hyperglycaemia. Encapsulated bacteria (Strep. The risk is increased in children under 5.20 corticosteroids should be given Correct Correct initial fluid resuscitation should be with normal saline the initial blood glucose level indicates the severity Correct Incorrect answer selected potassium often needs to be replaced Incorrect answer selected The treatment initially consists of fluid resuscitation with normal saline. Correct Is less common if splenectomy is for traumatic rupture. There is an increased risk of sudden overwhelming infection (sepsis or meningitis). haemolytic anaemia. The initial blood glucose is a poor indicator of severity.should be inserted and 20 ml/kg of N.0. Escherichia coli) are commonest. neisseria meningitidus. There is also an increased risk of malaria. replacement of potassium and treatment of the precipitating cause. immune cytopaenia. 17-Overwhelming septicaemia in post-splenectomy patients: True / False Can usually be prevented with prophylactic penicillin. Correct Because of the risk of post-operative sepsis. immune cytopaenias. Correct Is due to hypogammaglobulinaemia. saline given. Haemophilus influenzae. anatomic defects. The spleen is responsible for filtering the blood and for early antibody responses. Bicarbonate should be considered only if the pH is less than 7. These include: splenic rupture. splenectomy should be limited to specific indications. red cell membrane defects.

 SCID: Clinical spectrum of great genetic diversity where ID involves absence of T & B cell function from birth . MHC class I and II deficiency. with a vast number of underlying potential causes. 18-The following may indicate underlying severe combined immunodeficiency: True / False Platelet count of 40 Correct Absolute lymphocyte count of 0. but does not eliminate it. Examples of each category include:  Severe: Adenosime Deaminase Deficiency (ADA). In severe combined immune deficiency. and T cell defects.8 x 10 9/L Generalised lymphadenopathy Conjunctival telangiectasia Failure to thrive Incorrect answer selected Correct Correct Incorrect answer selected Severe combined immune deficiency is a clinical syndrome. In the case of trauma. and meningococcal A and C vaccines. Omenn's Syndrome. The appropriate duration of prophylaxis is unknown. X-SCID 1L2y deficiency. NK+ SCID. the child should be vaccinated with pneumovax. given the regulatory role of the CD4 cells and the cytokines they produce. Other clinical categories include combined immune deficiency. and preoperatively. ataxia telangiectasia. Hyper-IgE Syndrome. splenic repair or partial splenectomy may be possible. Post-splenectomy penicillin reduces the risk of pneumococcal sepsis. Hib. the immune deficiency involves the absence of T and B cell function from birth. where a secondary problem with immunoglobulin formation is almost inevitable. T+B+SCID.  Less Severe: Wiskott-Aldrich Syndrome. and purine nucleoside phosphorylase deficiency (PNP deficiency).The operation should be postponed until the patient is over 5.

Passive protection against measles should be given. A chest X-ray often shows a round lesion arising from the mediastinum. Immunisations up to date. No family or social history of note.Dissemination to the bone.7°C. The most likely diagnosis is a lymphoblastic lymphoma. with stony dullness to percussion and decreased breath sounds in the right base. Full term normal delivery. On examination the temperature is 36. 20-Which one of the following statements correctly applies to a child being treated for leukaemia? (Please select 1 option) Meningeal leukaemia is completely preventable Uric acid nephropathy occurs in remission Incorrect answer selected Male sex carries a favourable prognosis Passive protection with hyperimmune gammaglobulin should be given for measles This is the correct answer Treatment will usually result in infertility Prophylactic intrathecal chemotherapy and cranial radiation does not prevent meningeal leukaemia in all cases. Males have a worse prognosis and receive a longer duration of treatment. She looks unwell and has large rubbery enlargement of glands in the right lateral neck area. skin and CNS is not uncommon. Staging involves CT scans of the neck and thorax. no neonatal problems. respiratory rate 25/min and pulse 95/min. eg during chemotherapy. Uric acid nephropathy occurs when there are high levels of circulating blasts and cell breakdown is occurring. marrow. She is slightly breathless at rest.19-A 6-year-old girl presents with neck swelling and breathlessness. She has felt increasingly tired and unwell. as the immune response to the vaccine will be . worsening over 6 weeks. and detailed histology. What is the most likely diagnosis? (Please select 1 option) HIV infection Infectious mononucleosis Kawasaki disease Lymphoma Tuberculosis This is the correct answer Incorrect answer selected The history suggests pleural effusion and lymphatic infiltration.

a known diabetic. Henoch Schonlein Purpura Correct Henoch Schonlein purpura also known as anaphylactoid purpura has an unknown aetiology. Children present with abdominal pain. They may have a flitting arthritis affecting large joints. papular. . which do not disappear with pressure. Her mother. possibly melaena due to haemorrhage and oedema of the gut wall. He has swollen ankles and palpable lesions on his buttocks. The nephritis presents with microscopic haematuria and proteinuria and the macular. A 10-year-old girl has just returned from Thailand. She is brought to history of severe diarrhoea associated with abdominal pain.inadequate. however in males testicular relapse may occur. has similar symptoms. 21-Theme:Abdominal Pain A Acute gastro-enteritis B Appendicitis C Constipation D Diabetes Mellitus E Henoch Schonlein Purpura F Mesenteric Adenitis G Nephroblastoma H Pancreatitis I Sickle Cell crisis J Wilm's Tumour Select one option from the list above that is most suitable for the following patients A 12-year-old boy with a 3 week history of coryza is brought to A&E complaining of severe abdominal pain. Infertility is not common. purple rash is rather characteristic with buttocks and extensive surfaces of the legs and arms being affected.

. A 12-year-old girl with a 2 month history of weight loss and increased appetite weight loss is brought to Casualty with severe abdominal pain. Children present with polyuria. Correct Screening tests (Cochrane and Holland) should be:  Simple. Incorrect answer selected For haemaglobinopathies involves electrophoresis. Correct For neural tube defect has virtually eliminated the condition.  Repeatable.  Acceptable.  Accurate. In bacterial gastroenteritis. diarrhoea and possibly dehydration. Correct Incorrect answer selected For Down's syndrome has virtually eliminated the condition. She denies any history of vomiting or excessive exercise. 22-Regarding screening programmes: True / False For hypothyroidism uses B. polydipsia and polyphagia. vomiting. The commonest metabolic / endocrine problem in childhood. 60% of cases are due to viruses for example Rotavirus. In the developed world gastroenteritis is very common and usually mild. fever is common and children present with colicky abdominal pain. Children sometimes complain of abdominal discomfort and there is a history of weight loss. quick and easy to interpret. subtilis. For cystic fibrosis is universal. protein and fats. Diabetes Mellitus Correct describes a child with diabetes mellitus.Acute gastro-enteritis Correct relates to a child with gastroenteritis. Her mother says that the child has been wetting her bed over the past 4 months. Diabetes mellitus results from low Insulin levels resulting in abnormal metabolism of carbohydrate.

or using separate capillary specimens.  Hearing loss and visual impairment in 20%. the level of TSH at the time of diagnosis is an important prognostic marker. bacillus subtlis strains that grow only in high concentrations of phenylalanine. Closed forms continue to be born. The incidence is 2 per 1000 live births. Sensitive. The yield of a screening programme is defined as the number of new previously unsuspected cases per 100 cases screened. 23-Regarding cerebral palsy: True / False The incidence is 2 per 100 live births. About 3. The 2 most important haemoglobinopathies are sickle cell disease and thalassaemias. The clinical manifestations tend to evolve with age. In congenital hypothyroidism.  Squints in 30%. Epilepsy is present in 40%. Visual impairment occurs in 50%. If it is very high.  Specific. but it will soon become universal. Correct Incorrect answer selected Correct Correct Learning impairment is present in 30%.3% of the UK population belongs to a racial group at significant risk. Antibiotics may interfere with this test. They are thought to be extremely cost effective. Screening programmes for PKU and congenital hypothyroidism are well established. These include:  Learning impairment in 60%. impairment of neurological development may not be fully reversible. Hearing loss is present in 5%. Screening for PKU uses a bioassay.  Epilepsy in 40%. Screening for neural tube defects using alpha-fetoprotein has dramatically reduced the incidence of open spina bifida. and other problems are common and are reflecting more widespread damage to the brain. Screening should be universal in districts where the ethnic groups concerned make up more than 10% of the antenatal clinic population. Screening for Down's Syndrome using the triple test identifies approximately 60% of cases antenatally. Correct Cerebral palsy is a disorder of movement and posture due to a nonprogressive lesion of the motor pathways in the developing brain. The argument for screening for cystic fibrosis is less clear. Screening procedures usually involve either electrophoresis of a blood spot from the Guthrie card. .

ISDN). there may be considerable behavioural problems. In addition. 26-Theme:Presentation of infectious disease in childhood . MST. Marfan patients have mutations of the chromosome 15 gene encoding fibrillin. 24-Which of the following is conditions is associated with mutation in fibroblast growth factor receptor 3 (FGFR3) gene? (Please select 1 option) Retinoblastoma Achondroplasia This is the correct answer Neurofibromatosis type 1 Huntington disease Marfan syndrome Incorrect answer selected Mutations in the fibroblast growth factor receptor 3 cause achondroplasia. 25-A drug which undergoes high first pass metabolism True / False is excreted largely unchanged in the urine Correct can only be administered parenterally Correct is extensively metabolised by the liver Correct has a high extraction ratio Correct the extraction ratio is reduced in chronic liver disease Correct First pass hepatic metabolism implies extensive metabolism following absorption of from the gut. Speech and language disorders. Absorption of these agents is usually excellent (drugs may be lipo or hydro. Neurofibromatosis type 1 is caused by a mutation of the neurofibromin gene (which may act as a tumour suppressor) on chromosome 17q. a connective tissue protein. The extraction ratio depends upon liver function and is usually high (more drug removed following first pass).philic) and can be administered orally with increase of the dose (eg oestrogens.

Infectious mononucleosis Correct . The most common manifestation being an acute parotitis although severe infection with mumps may result in meningoencephalitis plus deafness. Commonly causes an acute gingivostomatitis. It is caused by a paramyxovirus and usually causes minimal symptoms. The virus is readily spread by direct contact especially to damaged skin e. eczema. for example a gingival stomatitis. Herpes simplex Correct Herpes simplex .the majority of children have benign manifestations of primary infection with Herpes simplex. Causing an exudative tonsillitis.A Chicken Pox B Herpes simplex C Infectious mononucleosis D Measles E Mumps F Mycoplasma G Pertussis H Rubella I Tuberculosis For each presentation of infectious disease choose the single most likely diagnosis from the list of options.g. Causing an acute parotitis Mumps Correct Mumps infection is now uncommon due to the vaccination.

4. but the disturbance not as excessive as anorexia nervosa. Correct .Glandular fever is also called infectious mononucleosis. Recurrent episodes of binge eating (rapid consumption of a large amount of food in a period of less than 2 hours). It is caused by EpsteinBarr virus and usually presents with an exudative pharyngitis or tonsillitis and cervical lymphadenopathy. Dental inspection may be helpful. 28-Medulloblastoma: True / False Can metastasise outside the CNS. A fear of not being able to stop eating during binges. 2. Hypokalaemia Correct Correct Correct Body mass index <16 Correct Abnormal perception of body size Incorrect answer selected Bulimia is defined in the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) as: 1. If abnormal then anorexia with bulimic features is the likely diagnosis. which is usually self limiting 27-Recognised findings in bulimia include: True / False Calluses on the dorsal surface of the hand. Self-evaluation unduly influenced by body weight and shape. or rigorous dieting or fasting to counteract the effects of binge eating. Their BMI is usually within the normal range. 5. Regular self-induced vomiting. use of laxatives. Correct Can metastasise down the neuroaxis. A minimum average of 2 binge eating episodes per week for at least 3 months. 3. It may cause a transient impairment of cellular and humeral immunity.

eyes. medulloblastoma. Gorlin Syndrome (nevoid basal cell carcinoma syndrome) is AD. mental retardation. spina bifida. endocrine system and bones.Are common in Gorlin's Syndrome. coloboma. hydrocephalus. It presents with ataxia plus raised intracranial pressure. 29-Theme:Mood disorders A Adjustment disorder with prolonged depressive reaction B Adjustment disorder with brief depressive reaction C Bipolar affective disorder D Depressive conduct disorder E Persistent depression with cyclothymia F Persistent depression with dysthymia G Post-schizophenic depression . CNS. Chemotherapy may be beneficial in completely resected cases and those with intraspinal metastases. and has a 5 year survival of 50%. Can respond to radiotherapy. kyphoscoliosis. glaucoma. Correct Can be successfully treated with chemotherapy. with absent or undescended testes.  Endocrine: hypogonadism. and up to 20% have spinal metastases at diagnosis. Treatment is with total CNS radiation after maximal surgical resection. blindness. Correct Correct Medulloblastoma accounts for 20% of brain tumours.  Eyes: cataract. strabismus. The tumour may seed throughout the CNS. It nearly always arises in the midline of the posterior fossa. fits. and via the CSF.  CNS: falx calcification. with a defect on chromosome 9.  Skin: early onset basal cell carcinoma. nerve deafness.  Bones: anomalous rib development. It includes a wide spectrum of defects involving skin. partial agenesis of the corpus callosum.

Major depression may be biologic or psychotic.H Recurrent depression with psychotic symptoms I Recurrent depression with somatic symptoms For each scenario choose the most likely diagnosis.g. e. A 13-year-old boy presents with feelings of worthlessness and decreased school performance. loss of interest in her hobbies. Adjustment disorder w ith prolonged depressive reaction Correct This boy is depressed following bereavement. In 50% of cases other conditions co-exist. so she has persistent depression with dysthymia. Both genetic (positive family history) and environmental factors may play a part. Depression is a mood disorder. His father committed suicide six months previously.  conduct disorder  anxiety  ADHD .The correct answer is Persistent depression with dysthymia This girl is depressed for no identifiable reason. Chronic depression lasts more than one year. so has an adjustment disorder with prolonged depression. Depressive conduct disorder Incorrect .The correct answer is Post-schizophenic depression This girl has primary schizophrenic symptoms and lowered mood. She feels that her teachers are picking on her all the time. poor concentration at school and weight gain of 10 kg over the past two and a half years. and she hears their voices criticising her for poor school performance. There are no "highs". A 12-year-old girl presents with irritability. Recurrent depression w ith somatic symptoms Incorrect . which ranges in severity. A 15-year-old girl presents following an overdose.

Trisomy 13 Correct Patau syndrome (trisomy 13) is associated with midline defects including hypotelorism. microphthalmia and cyclopia in some cases. 30-Theme:Large anterior fontanelles A Achondroplasia B Apert's syndrome C Athyrotic hypothyroidism D Hallermann-Streiff syndrome E Hypophosphatasia F Pyknodysostosis G Rubella syndrome H Russell-Silver syndrome I Trisomy 13 J Vitamin D dependent rickets Which of the listed conditions best fits the features below? Cleft palate and hypotelorism. . Apert's syndrome Correct Apert's syndrome is one of the craniosynostoses (premature fusion of the cranial sutures). school refusal. It is characterised by syndactyly of fingers and toes. cleft palate. Acrocephaly and syndactyly.

Hallermann-Streiff syndrome Incorrect . which can be confirmed by coeliac antibodies and biopsy. Gluten free diet Correct Likely coeliac disease. On examination he appears wasted. which mother feels has been brought on by the introduction of weaning.The correct answer is Hypophosphatasia Hypophosphatasia is a genetic abnormality of alkaline phosphatase. with loose buttock folds. which leads to a spectrum of bony disorders. . 31-Theme:Treatment of Diarrhoeal illness A Metronidazole B Gluten free diet C No action D Steroids E Cow's milk free diet F Pancreatic enzyme supplements G Vitamin D H High fibre diet I Breast milk allergy J Praziquantel Select one option from the list above that is most suitable for the following patients: A 6 month old baby has diarrhoea and failure to thrive. resulting in a fatal prognosis.A fatal metabolic condition of infancy associated with bony abnormalities and low serum alkaline phosphatase. and respond to gluten-free diet.

In Crohn's disease the colonoscopy will reveal cobbled stone mucosal appearances with fistulae where as in ulcerative colitis crypt abscesses are present. She has high anti-reticulin antibodies High fibre diet Incorrect . Steroids Correct relates to inflammatory bowel disease. He is also under the care of ophthalmologists for a chronic iritis.The correct answer is Gluten free diet refers to Coeliac disease where a gluten free diet is appropriate. The diagnosis is confirmed by jejunal biopsy. High reticulin or more specifically now anti-TTG antibodies are expected. She is Iron deficient and denies any history of GI upset after eating rice or potatoes.A 13-year-old Irish girl complains of diarrhoea. 32-Theme:WEAKNESS IN THE LOWER LIMBS A Guillain-Barre syndrome B Transverse Myelitis C Multiple sclerosis D Chronic fatigue syndrome E Spinal tumour F Dermatomyositis G Congenital myopathy H Duchenne muscular dystrophy I Fascioscapulohumeral dystrophy J Spinal abscess For these children with lower limb weakness select the most likely diagnosis . A 10-year-old boy presents with faecal urgency and bloody diarrhoea. Crohn's disease and ulcerative colitis respectively.

It is difficult to get her full cooperation for examination but there are no objective signs of weakness and reflexes are all present. On examination there is power with gravity eliminated in her legs. very brisk ankle and knee jerks and upgoing plantar responses. his muscles are well developed distally although weak proximally. urine culture urea & electrolytes were also normal. sore throat and cervical lymphadenopathy. He walked independently at 20 months and has received speech therapy for a mild speech delay. tiredness and weakness of her legs. Her bladder is palpable up to the umbilicus. vibration and position sense are preserved 3 weeks previously she had a presumed viral illness. She has been passing urine frequently and with difficulty. A blood count showed atypical lymphocytes and liver function tests show a raised AST. Speech delay. Lumbar puncture shows 33 lymphocytes and monocytes per microlitre and protein 570 mg/L in the CSF. Transverse myelitis has unknown aetiology but has been postulated to have a viral or mycoplasma origin. Sensation is reduced to touch below T5. A 12-year-old girl presents with a 5 day history of progressive weakness in her legs and is now unable to walk. MRI brain is normal but shows swelling and increased signal over the spinal cord T5-7 on T1weighted images. Chronic fatigue syndrome Correct The previous viral illness and subsequent lethargy with no consistent abnormal investigations suggest chronic fatigue syndrome. symmetrical with downgoing plantar responses. A 14-year-old girl has been off school for 4 months with headaches. cardiomyopathy. scoliosis and calf pseudohypertrophy are also features. In the initial weeks of her illness she had a low-grade fever. 33-The following are notifiable diseases in England and Wales True / False . Spinal abscess Incorrect . These were re-checked 1 month later when they had normalised and thyroid function. The CSF lymphocytosis and increased protein may be seen in transverse myelitis.The correct answer is Transverse Myelitis The features are suggestive of a lower motor neurone lesion which could also be caused by a spinal tumour / abscess however the MRI would demonstrate these latter pathologies. Duchenne muscular dystrophy Correct DMD occurs in boys and presents with delayed walking i. On examination his lower limb reflexes are normal.A 4-year-old boy is brought to clinic by his mother who feels he has weak legs and is finding climbing the stairs difficult.> 18months.e.

Chicken Pox is notifiable in Scotland. erysipelas. What is the most likely diagnosis? (Please select 1 option) Benign familial haematuria Haemolytic uraemic syndrome Haemorrhagic cystitis Correct .  Protozoal: malaria. measles. mumps. typhoid. On examination she is apyrexial. tetanus.rubella. Immunisations up to date. cholera. Respiratory rate is 15/min and pulse is 95/min. typhus. ophthalmia neonatorum. Brucellosis. meningitis. Blood pressure is 110/90 mmHg. food poisoning. followed by pallor. pertussis. leptosporosis.Acute encephalitis Dysentery Correct Correct Pulmonary tuberculosis Chickenpox Correct Correct Kawasaki Disease Correct Notifiable infections include:  Bacterial: anthrax. paratyphoid fever. varicella (encephalitis. meningococcal infection. scarlet fever. AIDS. tuberculosis. diphtheria. plague. hepatitis B. rabies. There is no FH/SH of note. Full term normal delivery with no neonatal complications. and reduced output of smoky coloured urine. poliomyelitis. leprosy. 34-A 3-year-old girl presents with a 3 day history of bloody diarrhoea. dysentery.chickenpox isn't) .  Viral: acute encephalitis. yellow fever. She appears pale and has a few petechiae over the abdomen.

with Escherichia coli 0157:H7. thrombocytopaenia and acute renal failure. Caused by a gram negative pleomorphic bacillus. 85% are associated with (bloody) diarrhoea. It typically presents with apnoeic episodes or cyanotic episodes during infancy. Pertussis Correct Whooping cough is not uncommon in infancy. and Shigella being the commonest culprits.Post-streptococcal glomerulonephritis Urinary tract infection The history of bloody diarrhoea followed by reduced output of blood-stained urine and petechiae strongly suggest Haemolytic Uraemic Syndrome. now the commonest cause of acquired renal failure in childhood. 35-Theme:Infections A Measles B Rubella C Chicken pox D Herpes simplex E Mumps F Glandular fever G Pertussis H Polio myelitis I Hepatitis A For each description below choose the single most likely infection from the list of options. This is the triad of intravascular thrombosis. Mumps . Is caused by a paramyxovirus which can cause orchitis. In the older child upper respiratory tract infections and a paroxysmal cough with a whoop is characteristic.

Correct Mumps and measles are caused by a paramyxoviruses. Unfortunately this is all too common these days. What is the most likely diagnosis? (Please select 1 option) Gastroesophageal reflux Allergic rhinitis Sinusitis Asthma Correct Croup A typical history of asthma in later childhood. 37-The following are recognised causes of post-neonatal hypoglycaemia: True / False Tricyclic overdose Urea cycle defects Correct Correct Congenital adrenal hyperplasia Incorrect answer selected Medium chain Acyl coenzyme-A dehydrogenase deficiency Correct . and admits to have started smoking. with exercise-induced symptoms and a general deterioration on commencement of smoking. Glandular fever Correct Glandular fever virus infects the B lymphocytes which results in an immunodeficiency which is usually self limiting. 36-A 14-year-old girl presents with a history of cough and breathlessness on exercise. Clinical examination is unremarkable. but only mumps causes orchitis. This infection is most often associated with post-viral fatigue. She has seasonal rhinitis.

MCAD deficiency. Abdominal pain and bloody stools may occur. exogenous insulin.Intravenous Salbutamol Correct The major causes are: METABOLIC:  a) Ketotic hypoglycaemia. The purpuric spots are vasculitic and do not blanch on pressure.  d) Poisoning: alcohol. acute liver failure. insulinomas.  d) Hyperinsulinsim: Nesidioblastosis. congenital adrenal HORMONAL: hyperplasia. . 38-In Henoch-Schönlein purpura: True / False The rash is usually on buttocks and extensor surfaces The platelet count is decreased Correct Correct Some patients develop minimal change nephrotic syndrome Incorrect answer selected Abdominal pain is common Correct The purpuric spots do not blanch on pressure Correct The rash is on the back and thighs. Beckwith's.  c) Inborn errors of metabolism (glycogen storage disease. aspirin.  a) Growth hormone. galactosaemia. organic acidaemia. It is a non thrombocytopenic purpura.  b) ACTH: panhypopituitarism.  b) Liver disease: Reye's Syndrome. hereditary fructose intolerance). The resulting glomerulonephritis is membranoproliferative rather than minimal change nephropathy. tyrosinaemia.  c) Cortisol: Addison's Disease.

68kg and there were no neonatal problems. and there is no FH/SH of note. recurrent Otitis media with effusion The history suggests acute otitis media with perforation. but some require surgical drainage 40-Marasmus: True / False . and her right ear is displaced downwards and forwards. Her pulse is 100/min and respiratory rate is 15/min. worsening over the past few days. which was treated with paracetamol. According to a study quoted in emedicine: "Abdominal pain was more common in children than adults (70. Pneumococcus and H. She is fully immunised.7C.2% vs 28. Symptoms began seven days ago with severe ear pain and fever. acute Otitis media. and has a purulent discharge from the ear canal. influenzae are the commonest cause.Abdominal pain is one of the cardinal symptoms of HSP. followed by acute mastoiditis (acute mastoid osteitis). This can be confirmed by CT scan of the mastoid. On examination she has a fever to 39. The following morning the pain resolved and a purulent discharge mixed with blood was noted on her pillow. Four days later the pain is throbbing and her ear is tender. Most resolve with antibiotics. She was born at term weighing 3.9%)" Henoch-Schönlein Purpura at eMedicine 39-A 7-year-old girl presents with right ear pain and fever. She is extremely tender behind the right ear. What is the most likely diagnosis? (Please select 1 option) Cholesteatoma Mastoiditis Correct Otitis media.

can coexist with kwashiokor. A baby is born with a pink appearance all commoner in twins Correct is due to protein deficiency Correct may be associated with severe chronic bowel disease the risk of superadded infection is high during recovery Correct Incorrect answer selected the recovery of older children is much greater than younger Correct Starvation. a respiratory rate of 30/min. The risk of infection is high at any time in fact if the child has no clinical sign of infection. Read more 41-Theme:Apgar score A 0 B 1 C 2 D 3 E 4 F 5 G 6 H 7 I 8 J 9 K 10 Select the most appropriate Apgar score for the following babies. a pulse of 125/min actively moving his limbs and is crying 10 Correct This is a full house: . the WHO recommends 5 days of oral cotrimoxazole therapy.

has some flexion of muscle tone.moving limbs (2)  respiration . The baby is evaluated on five simple clinical scores from 0-10.heart rate above 100 (2)  grimaces . A baby is born with a blue complexion. grimaces when stimulated with suction and has irregular respiration 5 Incorrect .The correct answer is 8 On this occasion:  appearance of blue peripheries (1)  pulse . has a heart rate of 106 on the monitor. crying (2). The Apgar score is a simple test to assess newborn health and was devised by Virginia Apgar in 1952.30/min normal. coughs when stimulated. A baby is born with peripheral extremities that are blue but is centrally pink.moving all four limbs (2)  respiration .more than 100 (2)  grimace .irregular (1).irregular respiratory effort (1). .crying (2)  activity .flexion (1)  respiration .The correct answer is 4 In this case:  appearance being blue (0)  pulse is less than 100 (1)  grimace when stimulated (1)  activity .coughs when stimulated (2)  activity . normal appearance . 7 Incorrect . a heart rate of 96/ (2)  pulse . moves all limbs and has some irregular respiratory effort.

LHRH agonists are used in the treatment of precocious puberty of all types and not clomiphene. There has been no vaginal bleeding: Which of the following is correct: (Please select 1 option) The diagnosis is unlikely to be precocious puberty Correct It is desirable to check her karyotype in making a diagnosis Lateral skull X-ray should be requested Menarche will likely take place in 3 years She will need to be treated with Clomiphene The diagnosis is unlikely to be precocious puberty as elevated gonadal steroid levels increase height velocity and the rate of skeletal maturation as well causing feminisation and breast development. The condition is more suggestive of premature thelarche. 43-Regarding osteosarcomas .8 years and height is on the 90th centile. an estrogen antagonist. 42-A girl aged 5 years attending normal school presents to the outpatient clinic with bilateral enlargement of the breasts. Karyotypic anomalies are not relevant. Appearance  Pulse  Grimace  Activity  Respiration. Puberty and menarche should take place at the normally expected timing( 11-13y). Bone age is 5. If central (LHRH dependent) precocious puberty is suspected then MRI would be of value rather than cranial ultrasound to identify hypothalamic lesions and other CNS lesions. However pelvic ultrasound showing normal uterine volume is the most sensitive discriminator between premature thelarche and true precocious puberty.

The onset is insidious with dyspnoea on exercise. with an increased FEV1:FVC ratio. There is no increase in airway resistance. Early haematogenous spread occurs and the five year survival rate is approximately 50%. They usually present as bone pain and a palpable lump. x Ray shows periosteal elevation (Codman's triangle) and a 'sunburst' appearance due to soft tissue involvement. The spirometry shows a restrictive defect. . later at rest. Gradually. chronic and often fatal disorder only occasionally seen in infants and children. Inspiratory crackles may be heard. weight loss. anorexia. They often occur in young adults but are also seen in the elderly in association with Paget's disease. There is an uncontrolled inflammatory process leading to progressive fibrosis. clubbing. They are most commonly seen around the knee and in the proximal humerus. compliance and diffusion capacity are decreased. Clinically.True / False Affects the epiphyses of long bones Correct Are most commonly seen around the knee and in the proximal humerus Correct Haematogenous spread can result in pulmonary metastases Is exclusively a disease of adolescence and early adult life Correct Correct x Ray shows a 'sunburst' appearance due to soft tissue involvement Correct Osteosarcomas affect the metaphyses of long bones. 44-Characteristic features of idiopathic diffuse interstitial fibrosis of the lung (HAMMAN-RICH) include: True / False Cyanosis on exercise Correct Inspiratory crackles on auscultation Hypercapnia Correct Incorrect answer selected Decreased FEV1/FVC ratio Correct Decreased gas transfer factor Incorrect answer selected This is a rare. but vital capacity. fatigue ability followed by cyanosis. there is progressive pulmonary insufficiency resulting from interstitial fibrosis and alveolar-capillary block. cor pulmonae and right sided heart failure occur.

It presents in early infancy. Is increased in emphysema. Correct Depends on the thickness of the alveolar wall. Diffusion will be increased in healthy compared with unhealthy lungs. the volume of gas diffusing across a membrane equals A/T x D x difference in partial pressure. the diffusion capacity for carbon monoxide. Correct Depends on the surface area available for gas exchange. and . VQ imbalances can indirectly interfere with carbon dioxide diffusion capacity by decreasing the available area of lung for gas exchange. In life it is impossible to measure accurately the area (A) or the thickness (T). where the thickness is likely to increase and the surface area available for gas exchange to decrease. Correct Incorrect answer selected Correct By Fick's law. Since the capillary blood normally does not contain carbon dioxide this term disappears. but it is not a specific measure of lung perfusion 46-The following are compatible with a diagnosis of Werdnig-Hoffmann Syndrome: True / False Fasiculation of the fingers Brisk tendon reflexes Incorrect answer selected Correct Pseudohypertrophy of the arm and leg muscles Incorrect answer selected Intercostal recession Correct Positive Gower's sign Correct Spinal muscular atrophy type 1 (Werdnig-Hoffmann Disease) is an autosomal recessive degenerative disorder of the anterior horn cells. There may be diminished fetal movements. and these are subsumed into a single constant. Is increased in cigarette smokers. DL=volume of transferred carbon dioxide divided by partial pressure difference between the alveoli and the capillary blood. which leads to progressive weakness and wasting of skeletal muscles.45-Diffusion capacity of carbon monoxide: True / False Is a specific measure of lung perfusion.

the seizure is generalised tonic clonic and lasts less than 10 minutes. roseola. Despite being very floppy. It is held partly flexed. may recur or be associated with a positive family history of epilepsy.9 C. the child is usually very alert. Atypical seizures last longer than 10 minutes. Typically. increasing to 9% when several risk factors are present. He had a full term normal delivery with no neonatal problems. He has previously been well. Copyright © 2002 Dr Colin Melville 48-A 2-year-old boy presents with fever and knee pain for 18 hours. He is alert and well perfused. an LP is indicated (if safe). red and exquisitely tender. and acute otitis media. Incorrect answer selected Febrile seizures occur in 3-4% of young children. Correct The risk of future epilepsy is 1%. occur below 9 months of age. lack of antigravity power and the hip flexors. The commonest precipitants are viral infections of the upper respiratory tract. may have focal features. There is no family or social history of note. His left knee is swollen. warm. The incidence of epilepsy in uncomplicated cases is 1%. Typical signs include fasiculation of the tongue. o . Correct An EEG is indicated if the fit is tonic clonic. particularly between 9 months and 5 years. 47-A 3-year-old girl has a fit in association with a temperature of 39°C: True / False A lumbar puncture is indicated. His immunisations are up to date. If any doubt exists with regard to the possibility of meningitis.arthrogryposis noted at birth. On examination the temperature is 38. Death is from respiratory failure by 12 months of age. Correct Fits lasting longer than 5 minutes should be thoroughly investigated. a febrile convulsion may signify a serious underlying acute infectious illness such as bacterial meningitis. with respiratory rate 24/min and pulse 100/min. or be associated with delayed development or abnormal neurological examination. Occasionally. and absent deep tendon reflexes. intercostal recession. Correct The commonest precipitating infection is a Streptococcal throat infection.

This can be confirmed by blood culture and needle aspiration of the joint. She has a dry mouth and reduced skin turgor.What is the most likely diagnosis? (Please select 1 option) Haemophilia A Juvenile rheumatoid arthritis Osteomyelitis Reactive arthritis Septic arthritis Correct The short history. with resolution followed by settling white cell count. Respiratory rate is 25/min and heart rate 100/min. This began two days ago. erythrocyte sedimentation rate and C reactive protein. The common organisms are Staph. Treatment is with intravenous antibiotics. with no previous medical problems.6C and is quiet. nor adverse FH/SH. No rash. with Guillain-Barré syndrome (GBS) in less than 3/12 infants. aureus and Gram negative Bacilli. The vomiting is now settling. location and clinical findings all point to a septic arthritis. On examination she has a temperature of 38. She was a healthy term infant. fever. but the diarrhoea has contained admixed blood and some slime. Her peripheries are cool and capillary refill time 4 seconds. 49-A 5-year-old girl presents with vomiting and diarrhoea. What is the most likely diagnosis? (Please select 1 option) Campylobacter gastroenteritis This is the correct answer Crohn disease Escherichia coli0157:H7 infection Salmonella typhi infection Viral gastroenteritis Incorrect answer selected .

50-A 18-year-old girl presents with anxiety and palpitations.4-5) Serum antithyroid peroxidase (anti TPO) titre 40 U/L (<50) What is the most likely cause of her symptoms? (Please select 1 option) Factitious thyrotoxicosis Familial hyperthyroglobulinaemia Hashitoxicosis Graves' disease Incorrect answer selected This is the correct answer Riedel's thyroiditis Although the lead-in might make you think that this patient could gain access to thyroxine and so a diagnosis of factitious hyperthyroidism is possible. A strong family history of thyrotoxicosis is typical for Graves' disease and the absence of a goitre with the absence of TPO antibodies (found in 80% of Graves' cases) again is compatible with a diagnosis of Graves'.This child has acute bloody diarrhoea (dysentery) accompanied by significant dehydration (5-10%). . Escherichia coli 0157:H7 causes occasional outbreaks and may be associated with haemolytic uraemic syndrome. On examination she had a pulse of 104 bpm with a fine tremor and lid lag. in practice this is extremely unlikely. The commonest offending organism is Campylobacter followed by Salmonella and Shigella. This is most likely due to a bacterial gastroenteritis. There was no goitre palpable. Occasional imported cases of typhoid are seen. Investigations revealed: Serum free T4 33 pmol/L (10-22) Plasma thyroid stimulating hormone (TSH) <0. Her mother had been treated for an overactive thyroid gland having received radioiodine and was now on thyroxine replacement therapy. and foreign travel is important to enquire about.05 (0. In this country undercooked frozen food (barbeques and takeaways) are the commonest culprits.