2004 FRACP Written Examination Paediatrics & Child

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2004 FRACP Written Examination

Paediatrics & Child Health

Paper 2 Clinical Applications

INTRODUCTION

Format

Paper 1 Medical Sciences: 70 questions; time allowed: 2 hoursPaper 2 Clinical Applications: 100 questions; time allowed: 3 hours

All questions are in the A-type multiple-choice format, that is, the single best answer of the five optionsgiven.

In the questions, values appearing within [ ] refer to normal ranges.

When visual material has been turned on its side, an arrow on the page indicates the orientation of thevisual material.

Questions do not necessarily appear in the order in which they were first printed.

AnswersA table of answers is located at the end of each paper.

ScoringA correct answer will score one mark and an incorrect answer zero. There is no negative marking in

the FRACP Written Examination.

QueriesContact the Executive Officer, Examinations Section, Department of Training and Assessment via e-mail: [email protected].

Please note that with changes in medical knowledge, some of the information may no longer becurrent.

Copyright 2005 by The Royal Australasian College of Physicians

All Written Examination papers are copyright. They may not be reproduced in whole or part withoutwritten permission from The Royal Australasian College of Physicians, 145 Macquarie Street, Sydney,

Australia.



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QUESTION 1

Which of the following vaccines would be most likely to be transmitted from a normal child to theirimmunocompromised sibling?

A. Bacille Calmette-Gurin (BCG).

B. Influenza.

C. Measles-mumps-rubella (MMR).

D. Oral polio.

E. Varicella-zoster.

QUESTION 2

An eight-year-old boy is admitted with an episode of acute asthma. His oxygen saturation is 92% in

air. He is prescribed prednisolone 1 mg/kg and hourly salbutamol 12 puffs. Three hours later hisoxygen saturation in air has dropped to 89%. He looks well and is less distressed than whenadmitted. He has widespread wheeze with good air entry.

Based on these findings, the most appropriate next step in his management is:

A. add ipratroprium bromide.

B. blood gas analysis.

C. change to intravenous salbutamol.

D. chest X-ray to rule out a pneumothorax.

E. reduce frequency of salbutamol.

QUESTION 3

A 13-year-old boy presents with nonspecific abdominal pain. The boy has a history of seizures. Hismother is known to have chronic renal failure. Physical examination shows a small 13-year-old who isnormotensive with small areas of hypopigmentation on his trunk. The abdominal pain quicklysubsides following hospital admission. A renal ultrasound scan shows enlarged kidneys and multipleechogenic foci throughout both kidneys.

Which one of the following is the most likely explanation of the renal abnormality in this boy?

A. Dominant polycystic kidney disease.

B. Juvenile nephronophthisis.

C. Papillary necrosis.

D. Recessive polycystic kidney disease.

E. Renal angiomyolipomata.



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QUESTION 4

When assessing an adolescent, whose history is likely to have the greatest sensitivity for the detectionof depressive symptoms?

A. Friends.

B. Siblings.

C. The adolescent.

D. The classroom teacher.

E. The mother.

QUESTION 5

A 15-year-old girl is brought into the emergency room by friends after attending a birthday party. Her

friends are concerned because there has been an alteration in her conscious state. She is febrile andis visibly flushed and sweating. Her heart rate is 120/minute and regular. Her blood pressure is160/95 mmHg. Her neurological exam was notable for her being combative with small but reactivepupils. During your examination she begins to have a generalised seizure.

She has a history of depression and is being managed by a psychiatrist.

You suspect an ingestion or intoxication.

Which of the following is the most likely cause?

A. Alcohol.

B. Ecstasy.

C. Marijuana.

D. Mushroom intoxication.

E. Tricyclic antidepressants.

QUESTION 6

A two-year-old boy is taken to the emergency department by his parents with rectal bleeding. Hepassed about 100 mL of blood per rectum. The blood is slightly altered. There were no associatedabdominal pains, and the abdomen is soft and non-tender on examination. He has no cutaneousstigmata or telangiectasia.

Which of the following is the most likely diagnosis?

A. Campylobacterinfection.

B. Inflammatory bowel disease.

C. Intussusception.

D. Meckel diverticulum.

E. Polyp.



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QUESTION 7


Parasternal long axis echocardiogram

Apical 4-chamber echocardiogram

Key: AO aorta

RA right atriumLA left atriumLV left ventricleRV right ventricle

The echocardiograms shown above were obtained from an otherwise healthy three-month-old infantpresenting for assessment of a murmur. They show multiple intraventricular lesions.


A. Neurofibromatosis type 1.

B. Noonan syndrome.

C. Proteus syndrome.

D. Sturge-Weber syndrome.

E. Tuberous sclerosis.


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QUESTION 8

A five-year-old child has the karyotype shown above. What is the most likely phenotype at this age?

A. Ambiguous genitalia.

B. Developmental delay.

C. Normal female.

D. Normal male.

E. Short stature.

QUESTION 9

Unawareness of hypoglycaemia is predominantly due to failure of secretion of which one of thefollowing hormones?

A. Adrenaline.

B. Cortisol.

C. Glucagon.

D. Growth hormone.

E. Somatostatin.



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QUESTION 10

A two-year-old boy from Nigeria immigrated a year ago. He presents to the emergency departmentwith a fever of 40C. His mother says that he has known sickle cell disease diagnosed previously inAfrica. Initial examination reveals a sick child along with an initial full blood count showing a raisedneutrophil count with left shift.

The most likely organism causing his fever is:

A. Epstein-Barr virus.

B. Haemophilus influenzaetype b.

C. Plasmodium falciparum.

D. Salmonella typhi.

E. Streptococcus pneumoniae.

QUESTION 11

A three-year-old girl is found floating face down in the family pool after being missed forapproximately five minutes. Basic life support is commenced on the scene and on arrival in theemergency department the girl is in asystole. She is apnoeic and pulseless, her temperature is 33Cand the maximum estimated period of pulseless apnoea is 15 minutes. The child is rapidly intubatedand ventilated, intravenous access is gained and external cardiac compressions are continued.

Which of the following is the most appropriate next step in the acute management of this child?

A. Administer intravenous adrenaline.

B. Administer intravenous atropine.

C. Commence active warming measures.

D. Counsel the family and cease active resuscitative measures.

E. Immediate direct current (DC) defibrillation.

QUESTION 12

Which of the following is least consistent with night terrors?

A. Amnesia of the episode by the child.

B. Family history.

C. Occurrence in rapid eye movement (REM) sleep.

D. Occurrence in the first third of the night.

E. Onset at five years of age.



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QUESTION 13

A 41-week gestation female infant was born by normal vaginal delivery with a birth weight of 3045 gand head circumference of 33.5 cm. A rash on her limbs was noted on the first day of life. Sheestablished breastfeeding very well. At 5 days of age, she was noted to be irritable and feedingpoorly. She developed left sided clonic jerks with eye deviation. These were controlled with

intravenous phenobarbitone.


The rash as illustrated above was described as an erythematous vesicular rash which was crusted inplaces. It was present on the forearms and lower limbs.

The most likely cause of this rash would be:

A. congenital bullous ichthyosiform erythroderma.

B. congenital varicella.

C. epidermolysis bullosa.

D. herpes simplex virus infection.

E. incontinentia pigmenti.


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QUESTION 14

The flow-volume loop shown above is most consistent with:

A. aberrant right subclavian artery.

B. asthma.

C. laryngeal web.

D. subglottic stenosis.

E. tracheomalacia.



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QUESTION 15

A 24-year-old pregnant woman reports at her first antenatal visit that her sister gave birth to a childwith congenital heart block (CHB). Which one of the following tests would be most useful in assessingthe risk of CHB in this pregnancy?

A. Antibodies to DNA.

B. Antibodies to extractable nuclear antigens (ENA).

C. Anticardiolipin antibodies.

D. Fluorescent antinuclear antibody (ANA) test.

E. Lupus anticoagulant.

QUESTION 16

A six-month-old boy presents with a two-month history of persistent vomiting, loose bowel motions,poor weight gain, irritability after feeds and a persistent rash on his face and upper arms. He was bornat term, breastfed to two months of age, and subsequently changed to a cows milk-based formula.Solids, including a wheat-based cereal, had been introduced from four months of age.

The following immunological investigations were performed:

total serum IgE 7 kU/L [0-20]

total serum IgA 0.37 g/L [0.14-0.69]

radioallergosorbent assay to cow's milk


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QUESTION 17

A mother with a history of genital herpes delivers a baby at term by normal vaginal delivery. Threedays after birth the infant is well, but is noted to have two small blistering lesions on the arm.Investigations confirm herpes simplex virus type 2 from the skin lesions.

The most appropriate treatment would be:

A. intravenous aciclovir.

B. intravenous ganciclovir.

C. observation.

D. oral aciclovir.

E. oral famciclovir.

QUESTION 18

An eight-year-old child presents to a regional emergency department with a one-week history ofweakness in both legs, stumbling gait and complaints of numbness. The family is currently underconsiderable stress with the suicide of a close relative and parental fighting. Neurological examinationreveals apparent reduction in power, variable sensory loss and normal deep tendon reflexes. The gaitis erratic.


A. Conversion disorder.

B. Guillain-Barr syndrome.

C. Multiple sclerosis.

D. Transverse myelitis.

E. Viral myositis.

QUESTION 19

The occurrence of a second malignancy after successful treatment of a primary tumour is least likelyto be associated with:

A. Bloom syndrome.

B. cyclophosphamide.

C. Fanconi aplastic anaemia.

D. growth hormone treatment.

E. radiotherapy.



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QUESTION 20

A 10-year-boy presents with an episode of acute left-sided loin pain. He passes bright red urine andrequires intravenous morphine for analgesia. A plain abdominal X-ray is shown below.

Which one of the following is most likely to play an important role in the development of this condition?

A. High sodium diet.

B. Hypokalaemia.

C. Hypomagnesaemia.

D. Increased urine citrate concentration.

E. Metabolic alkalosis.



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QUESTION 21

A five-month-old baby boy returns for review of his congenital hypothyroidism. He continues to growalong the 50th percentile for his length, weight and head circumference. He has been on anappropriate dose of daily replacement with thyroid hormone (37.5 g/day).

Repeat thyroid function tests (carried out at his visit) are as follows:

thyroxine (T4) 18 pmol/L [10-23]

thyroid-stimulating hormone (TSH) 153.0 mU/L [0.4-4.0]

The most likely explanation for the elevated TSH is:

A. expected result, as TSH can take many months to suppress.

B. iodine deficiency.

C. malabsorption.

D. poor parental compliance.

E. thyroid hormone resistance.

QUESTION 22

A seven-year-old boy wakes in the morning and cries out to his parents to come quickly. Hecomplains to his parents that the room is spinning. The symptoms seem to be aggravated by headmotion and change of position from lying down. He becomes pale, sweaty and nauseated wheneverhe moves or tries to sit up in bed. He is helped to the bathroom because he has difficulty standingand walking unsupported and vomits repeatedly. His parents bring him to the emergency department

soon afterwards.

On examination, he is found to be perfectly conscious and cooperative if left to lie still in bed. He hasleft-beating nystagmus in all positions of gaze but otherwise no focal neurologic findings. His hearingis normal. He has a mild upper respiratory tract infection.

The most likely diagnosis is:

A. acute disseminated encephalomyelitis (ADEM).

B. acute labyrinthitis.

C. acute vestibular neuritis.

D. cerebellar ataxia.

E. Menires disease.



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QUESTION 30

A seven-year-old girl presents with a history of bleeding gums after she cleans her teeth. Examinationof her mouth reveals evidence of gingivitis as shown below.

A defect of which component of immune function is most likely to be associated with this clinicalpresentation?

A. B cells.

B. Complement.

C. Natural killer cells.

D. Neutrophils.

E. T cells.

QUESTION 31

A four-year-old boy with severe haemophilia A and a high titre factor VIII inhibitor needs surgery. Theperi-operative replacement coagulation product of choice is:

A. cryoprecipitate.

B. fresh frozen plasma.

C. recombinant activated factor VII.

D. recombinant factor VIII.

E. recombinant factor IX.



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QUESTION 32

An 11-year-old boy presents to the emergency department following a fall onto his outstretched leftarm whilst rollerblading. On examination his left hand is neurovascularly intact and there is nodeformity. His X-ray is shown below.

Which of the following is the most appropriate next step in management?

A. Apply backslab and review in one week.

B. Closed reduction in the emergency department.

C. Closed reduction in the operating theatre.

D. Open reduction.

E. Urgent orthopaedic review in the emergency department.



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QUESTION 33

A three-month-old boy, 10% dehydrated with rotavirus diarrhoea, has a serum sodium of 166 mmol/L[135-145]. He is normotensive, has just passed urine and when weighed two days ago was 5 kg.Intravenous fluid is commenced.

Which of the following is the most appropriate regimen?

A. 0.18% saline, 4% dextrose and 20 mmol/L potassium chloride at 35 mL/hour.

B. 0.18% saline, 4% dextrose and 20 mmol/L potassium chloride at 50 mL/hour.

C. 0.45% saline, 2.5% dextrose and 20 mmol/L potassium chloride at 50 mL/hour.

D. 0.9% saline and 20 mmol/L potassium chloride at 35 mL/hour.

E. 0.9% saline and 20 mmol/L potassium chloride at 50 mL/hour.

QUESTION 34

A 12-year-old boy presents with chafed and reddened hands as a result of frequent washing. Hisfather is an infectious disease physician. The boy describes intrusive thoughts about infection withPseudomonas and scrupulously cleans the family bathroom each time before using it. He isconcerned about his father bringing home contaminants and has become increasingly isolated fromother members of the family.

The best treatment will be multimodal. The component with the best evidence of efficacy is:

A. clomipramine.

B. cognitive-behaviour therapy.

C. family therapy.

D. sertraline.

E. venlafaxine.

QUESTION 35

A nine-month-old boy presents after a three-minute tonic-clonic seizure, associated with a fever of39C. He is fully immunised. The infant has a number of small ulcers on the tip of the tongue and onthe gingival mucosa of the lower incisors. The following day the infant develops weakness of his leftleg.

The infant is most likely to be infected with:

A. cytomegalovirus (CMV).

B. enterovirus 71.

C. Epstein-Barr virus (EBV).

D. human herpes virus 6 (HHV-6).

E. poliovirus type 3.



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QUESTION 36

A 14-year-old boy complains of heel pain of six months duration. The following X-ray is obtained.

Which of the following is the most likely cause of his heel pain?

A. Ankylosing spondylitis.

B. Calcaneal osteomyelitis.

C. Hypermobile pes planus.

D. Reflex sympathetic dystrophy.

E. Sever disease (Achilles tendonitis).

QUESTION 37

A term newborn infant is noted to be tachypnoeic from delivery. On examination on day 1 he is alsonoted to have prominent pulses and an active precordium. His liver is palpable 4 cm below the costalmargin. No murmurs are audible. His arterial oxygen saturation is 98% in room air.


A. Aneurysm of the vein of Galen.

B. Group B streptococcal sepsis.

C. Hypoplastic left heart.

D. Patent (persistent) ductus arteriosus.

E. Thyrotoxicosis.



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QUESTION 38

A 26-year-old woman has a baby with Down syndrome. Subsequent investigations reveal that thewoman is a carrier of a balanced Robertsonian translocation involving chromosomes 14 and 21. Herkaryotype is 45,XX,t(14;21). In a subsequent pregnancy, she declines the offer of prenatal testing.

If the pregnancy goes to term, the most likely outcome is a baby with:

A. an attenuated form of Down syndrome due to mosaic trisomy 21.

B. Down syndrome.

C. intrauterine growth retardation due to maternal isodisomy 14.

D. normal phenotype.

E. severe malformations due to trisomy 14.

QUESTION 39

A 12-month-old girl is referred by her general practitioner because of failure to thrive. Her weight wason the 50th percentile at six months but had fallen to the 10th percentile at one year. She had a moistcough at nine months which lasted 10 days. Neonatal immunoreactive trypsin screening wasnegative.

On examination she has buttock and thigh wasting. A full blood examination reveals a mildleucocytosis and her electrolyte profile demonstrates the following:

serum sodium 120 mmol/L [135-145]

serum potassium 2.0 mmol/L [3.5-5.0]

serum chloride 90 mmol/L [90-110]

The most appropriate next investigation is:

A. anti-endomysial antibody.

B. serum and urinary osmolality.

C. serum 17-OH progesterone.

D. sweat test.

E. urinary sodium and potassium.



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QUESTION 40



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QUESTION 40 (continued)

An eight-year-old girl is referred to hospital because of progressive pallor for three months. In the pastfour weeks, she has developed painful lesions on her feet, as shown opposite. There is no pastmedical history of note.

Initial investigations show the following:

haemoglobin 89 g/L [110-140]

white cell count 6 x 109 /L [4-11]

neutrophils 4.0 x 109 /L [1.4-4.6]

lymphocytes 1.0 x 109 /L [1.4-4.6]

eosinophils 0.20 x 109 /L [


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QUESTION 42

A 13-year-old girl presents to your office with a six-month history of daily headaches. The headachesare present on waking and on occasions are associated with vomiting without nausea. She has hadintermittent double vision and transient episodes of loss of vision lasting seconds only. She is takingtetracyclines for acne.

On examination, she is moderately obese, afebrile, and has no neurocutaneous stigmata. She has amoderate amount of facial acne. Her blood pressure is 105/65 mmHg. Her extraocular movementsshow a partial left sixth nerve palsy and she has gross papilloedema. There are no other abnormalfindings.

A T2-weighted magnetic resonance imaging (MRI) scan of her brain is shown below.


A. aqueductal stenosis.

B. benign intracranial hypertension (BIH).

C. craniopharyngioma.

D. meningioma.

E. temporal lobe tumour.



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QUESTION 43

A nine-year-old girl is seen with polyuria and polydipsia. She is dehydrated and on routineassessment is found to have the following blood test results:

glucose 38.2 mmol/L [3.8-6.2]

sodium 130 mmol/L [134-143]potassium 4.2 mmol/L [3.5-5.6]

pH 7.12 [7.35-7.45]

bicarbonate 9 mmol/L [18-29]

creatinine 0.035 mmol/L [


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QUESTION 45

A breastfed four-week-old girl is taken to hospital because of several episodes of rectal bleeding withmost bowel motions. The infant is passing four stools daily with visible streaks of bright red blood andmucus. The infant is otherwise well and thriving.

Stool testing reveals the following findings:

red blood cells +++

white blood cells +

fat globules +

fatty acid crystals negative

reducing sugars 0.25%


A. Allergic proctocolitis.

B. Anal fissure.

C. Bacterial gastroenteritis.

D. Juvenile polyp.

E. Swallowed maternal blood.

QUESTION 46

The distribution of joint involvement indicated by the red colour in the diagram above is mostcharacteristic of which form of childhood arthritis?

A. HLA B27-associated arthropathy.

B. Polyarticular juvenile idiopathic arthritis.

C. Psoriatic arthritis.

D. Rheumatic fever.

E. Systemic lupus erythematosus.



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QUESTION 47

Diagnosis of a major depressive disorder according to the Diagnostic and Statistical Manual, 4thedition (DSM-IV) requires a number of symptoms to have been present nearly every day during thesame two-week period.

Which of the following symptoms is least helpful in the diagnosis of adolescent depression?

A. Anhedonia (feeling of loss of pleasure in all, or almost all, activities).

B. Depersonalisation (feeling of being detached from oneself).

C. Impaired concentration.

D. Irritable mood.

E. Marked change in appetite.

QUESTION 48

Serological evidence of recent streptococcal infection is least likely to be associated with which of thefollowing manifestations of rheumatic fever?

A. Carditis.

B. Erythema marginatum.

C. Migratory polyarthritis.

D. Subcutaneous nodules.

E. Sydenham chorea.

QUESTION 49

Infusion of cryoprecipitate is principally indicated for the replacement of:

A. anti-thrombin.

B. factor IX.

C. fibrinogen.

D. IgG.

E. protein C.



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QUESTION 50

An 11-year-old boy is admitted to hospital because he has been lethargic for the past three to fourweeks. He appears to be thirsty all the time with recent weight loss. He has been getting up to passurine three times each night for the past two weeks. There is no history of drug ingestion or infection.There is no past history of note. Clinical examination is unremarkable.

His initial investigations show the following:

serum:

sodium 135 mmol/L [135-146]

potassium 3.0 mmol/L [3.5-5.0]

urea 16.0 mmol/L [3.2-7.7]

creatinine 0.29 mmol/L [0.04-0.08]

glucose 3.5 mmol/L [2.5-7.0]

urinalysis 1+ protein, no blood, moderate glucose

urine microscopy 40 white cells,


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An eight-month-old girl presents with a history of cough and recurrent chest infections. A clinicalphotograph and chest X-ray of the patient are shown opposite.

Which one of the following is the most likely cause of this infants clinical presentation?

A. Lymphoma.

B. Neuroblastoma.

C. Retinoblastoma.

D. Sarcoidosis.

E. Thymoma.

QUESTION 54

An 11-year-old boy has the facial appearance shown above. He has a history of cleft palate repairand has borderline developmental delay.


A. Fetal alcohol syndrome.

B. Fragile X syndrome.

C. Kabuki make-up syndrome.

D. Myotonic dystrophy.

E. Velocardiofacial syndrome.



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QUESTION 55

Following a normal pregnancy a male infant has a normal vaginal delivery at term in the presence ofclear liquor. Immediately following delivery, he is in severe respiratory distress with marked subcostaland sternal recession. A normal cry is heard. A soft, high-pitched, biphasic stridor is audible.

The most likely cause of this infants respiratory distress is:

A. bilateral abductor vocal cord paralysis.

B. laryngo-oesophageal cleft.

C. lingular cyst.

D. severe laryngomalacia.

E. unilateral vocal cord paralysis.

QUESTION 56

A 14-month-old boy is brought to see you by his mother for advice about his diet and which foodsshould be avoided because of his allergies. At seven months of age, following his first exposure toegg, he developed perioral urticaria within 10 minutes which resolved after 15 minutes. There were noother symptoms. At 12 months of age, 5 minutes after ingestion of a small amount of peanut butter ontoast, he developed generalised erythema, cough and seemed distressed. No medical attention wassought and the symptoms resolved spontaneously over 30 minutes. He has not had egg or peanutsince these reactions and has not been exposed to other nut products. He drinks soymilk because hismother believes this is better for allergies than cows milk, however he can eat yoghurt and ice creamwithout any obvious symptoms. He had atopic dermatitis on the face and flexures until 12 months ofage but this has resolved. Allergen skin prick tests show:

Allergen Wheal diameter (mm)Cows milk 5Egg white 0Peanut 9Cashew nut 9Soy 3

Histamine control 5Saline control 0

On the basis of the history and the results of skin prick tests the most appropriate advice would be toavoid:

A. peanut alone.

B. peanut and cashew nut.

C. peanut, cashew nut and cows milk.

D. peanut, cashew nut, cows milk and soy.

E. peanut, cashew nut, cows milk, soy and egg.



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QUESTION 57

A five-year-old girl is brought to her paediatrician because of recurrent headaches. During thephysical examination a number of skin lesions were found. These are demonstrated in thephotograph below.

Which of the following is the least likely to be associated with her condition?

A. Acoustic neuroma.

B. Learning difficulties.

C. Macrocephaly.

D. Pseudoarthrosis.

E. Scoliosis.

QUESTION 58

An infant is noted on day 2 of life to be cyanosed. The arterial oxygen saturation is 88% in room air.The remainder of the examination is normal, including normal cardiac and respiratory examinations.


A. Alveolar capillary dysplasia.

B. Extralobar sequestration.

C. Tetralogy of Fallot.

D. Totally anomalous pulmonary venous return.

E. Transposition of the great arteries.



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QUESTION 59

A four-year-old boy is referred to you for investigation of persistent diarrhoea and poor weight gain.The following results are obtained:


mean corpuscular volume (MCV) 70 fL [75-90]total serum IgA 0.07 g/L [1.23-2.32]

antigliadin-IgG antibody 88 U/L [


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QUESTION 62

A 10-year-old boy is referred with concerns regarding his behaviour. His teachers are concerned thathe is easily distracted, has a poor attention span and appears to have low self-esteem. He oftenappears blank and seems to miss instructions. Over the past six months he has been increasinglydefiant towards teachers, and he has begun acting the class clown. He has had two detentions this

term for inappropriate behaviour.

At home he has been moody and at times aggressive toward his parents. He was an easy child untilhe was seven or eight years old, but has been increasingly unhappy since. He has never reallyenjoyed school.

He had language delay identified at age three, and had some speech therapy. Otherwise hisdevelopmental milestones were achieved age-appropriately.

The school arranged psychometric testing. On the Wechsler Intelligence Scale for Children-ThirdRevision he had a full-scale intelligence quotient (IQ) of 82, with a performance scale score of 104 anda verbal scale score of 72. On academic achievement testing his arithmetic score was within theaverage range, but his reading and spelling scores were both more than two standard deviations

below the mean. His literacy level was assessed at two years below his current grade level. Theeducational psychologist noted that he was reluctant to attempt some tasks.

Which of the following is the most likely primary diagnosis?

A. Attention deficit/hyperactivity disorder.

B. Depression.

C. Intellectual disability.

D. Primary generalised epilepsy with absence seizures.

E. Specific learning disability.

QUESTION 63

A four-year-old girl with Alagille syndrome (intrahepatic hypoplasia of bile ducts) and long-standingcholestatic jaundice presents with a broad-based ataxic gait. On examination her tendon reflexes areabsent.

Which of the following is the most likely cause?

A. Folate deficiency.

B. Hypothyroidism.

C. Posterior fossa tumour.

D. Vitamin B12 deficiency.

E. Vitamin E deficiency.



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QUESTION 64

An eight-year-old boy presents with a two-month history of fever, night sweats, abdominal pain, achingjoints and weight loss of 2 kg. He has developed the rash shown above over the last two weeks. Hewas previously well.

Examination shows the rash but no other significant clinical findings.


A. Dermatomyositis.

B. Henoch-Schnlein purpura.

C. Parvovirus infection.

D. Polyarteritis nodosa.

E. Tuberculosis.

l Australasian College of PhysiciansCopyright 2005 by The Roya


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QUESTION 65

A 14-year-old girl complains of tiredness. She has no significant past medical history and denies anymedications. Apart from being thin, there are no abnormal findings on examination. Her bloodpressure is 105/70 mmHg.

Results of investigations are listed below.

Serum biochemistry:

sodium 138 mmol/L [135-145]

potassium 2.3 mmol/L [3.4-5.0]

chloride 85 mmol/L [103-109]

creatinine 0.10 mmol/L [0.06-0.12]

Arterial blood gases:

pH 7.50 [7.34-7.45]

PaO2 95 mmHg [80-100]PaCO2

42 mmHg [35-45]

bicarbonate 39 mmol/L [22-28]

Urinary biochemistry:

sodium 30 mmol/L

potassium 42 mmol/L

chloride 13 mmol/L

The most likely explanation for these results is:

A. Bartter syndrome.

B. occult diuretic use.

C. occult laxative use.

D. primary hyperaldosteronism.

E. self-induced vomiting.

QUESTION 66

An 18-month-old child presents after swallowing a 20-cent piece. At which of the following anatomicalsites is the foreign body most likely to become impacted?

A. Duodeno-jejunal junction.

B. Gastro-oesophageal junction.

C. Ileocaecal valve.

D. Oesophagus at level of cricoid.

E. Pylorus.



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QUESTION 67

A four-year-old boy presents with worsening back pain, which is more severe at night. The pain firststarted about three weeks previously after a minor upper respiratory infection and was initiallyintermittent. There has not been any recent history of fever. He had previously been well, with nosignificant infections other than otitis media.

Examination reveals tenderness over the lumbar spine at L2/3, limited straight leg raising and a loss ofthe normal lumbar lordosis. His erythrocyte sedimentation rate (ESR) is 60 mm/hr and his C-reactiveprotein is 4 mg/L [


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QUESTION 69

The most common adverse effect of methylphenidate is:

A. appetite suppression.

B. headache.

C. sleep difficulty.

D. tics.

E. withdrawn behaviour.

QUESTION 70

The parents of a two-year-old boy seek advice about their past reproductive and family history. Theyhave recently immigrated from Vietnam. The pedigree is as shown below. There have been four

miscarriages, all between six and eight weeks of gestation. Their first child (III:4) was born withmultiple congenital anomalies and had severe developmental delay. He died aged two years, ofunspecified causes. No other information is available about him. The only other family history of noteis that the deceased childs uncle (II:1) also had developmental delay and died in childhood. Thesurviving child (III:6) is normal.

Which of the following investigations is most likely to prove useful for counselling about futurechildren?

A. Haemoglobin electrophoresis of both parents.

B. Karyotypes of both parents.

C. Maternal fragile X triplet repeats.

D. Maternal lupus serology.

E. Maternal plasma very long chain fatty acids.



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QUESTION 71

A six-month-old girl presents with a short history of fever (38C) and exacerbation of her facialeczema, as shown in the photograph above. She has a strong family history of asthma.

Initial treatment with oral flucloxacillin and topical 1% hydrocortisone ointment shows no improvement.

Which of the following is most likely to improve her condition?

A. Intravenous aciclovir.

B. Intravenous amoxycillin/clavulanic acid.

C. Intravenous flucloxacillin.

D. More potent topical steroids.

E. Wet saline dressings.

QUESTION 72Which of the following symptoms or signs would be least likely in a child with Kawasaki disease?

A. Arthritis.

B. Erythema of buttocks.

C. Jaundice.

D. Purulent conjunctivitis.

E. Right hypochondrial tenderness.



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QUESTION 74

Antenatal maternal smoking is least associated with which one of the following effects in children?

A. Decreased lung compliance.

B. Decreased maximal expiratory flow.

C. Increased risk of lower respiratory tract infections.

D. Increased risk of sudden infant death syndrome.

E. Increased risk of wheezing.

QUESTION 75

A 10-year-old girl with a longstanding history of migraine, on no medications, presents with severeheadache and left upper limb weakness.

Which of the following medications should not be used at this point?

A. Codeine.

B. Cyproheptadine.

C. Dihydroergotamine.

D. Ibuprofen.

E. Propranolol.

QUESTION 76

A previously well six-year-old girl presents for assessment of pubertal development. On examinationher height is above the 97th percentile and her weight is on the 25th percentile. She has Tannerstage 3 breast development. Pubic hair is Tanner stage 1. She has no skin markings. Investigationshows her bone age is equivalent to that of an eight-year two-month-old girl.


A. Adrenal tumour.

B. Congenital adrenal hyperplasia.

C. Ovarian cyst.

D. Pituitary hamartoma.

E. Premature thelarche.



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QUESTION 77

A five-year-old girl presents with a two-day history of fever to 38C axillary and a swollen, tender rightknee. She has previously been well, and has had no rash and no previous episodes of joint pain. Onexamination, she is febrile (37.9C) and flushed. She has a heart rate of 100/minute with normal heartsounds. She has shotty cervical lymphadenopathy, but no palpable liver or spleen. Her right knee is

warm, swollen, red, and slightly tender. She has no effusion clinically. She can straighten her leg andflex the right knee, but there is pain at the extreme of knee flexion.

Her blood test results are as follows:


white cell count 10.8 x 109 /L [4.0-11.0]

differential:

neutrophils 3.5 x 109 /L [1.0-4.0]

lymphocytes 7.3 x 109 /L [3.0-8.0]

platelet count 170 x 109 /L [150-400]

erythrocyte sedimentation rate (ESR) 18 mm/hr [


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QUESTION 79

A one-week-old full term baby girl is noted to be tachypnoeic. Her chest X-ray is shown below.


A. cardiac failure.

B. congenital cystic adenomatous malformation.

C. congenital lobar emphysema.

D. diaphragmatic hernia.

E. staphylococcal pneumonia.



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QUESTION 80

A nine-year-old boy presents with a two-week history of cough, tachypnoea, recession and increasing

cyanosis. The chest X-ray and a lung biopsy (stained with methenamine silver nitrate) are shownabove.

The underlying immune deficiency is most likely to be primarily affecting his:

A. B lymphocytes.

B. macrophages.

C. natural killer cells.

D. neutrophils.

E. T lymphocytes.



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QUESTION 81

A newborn is receiving assist-control ventilation with the following ventilator settings:

peak inspiratory pressure 17 cmH2O

positive end-expiratory pressure 4 cmH2O

back-up ventilator rate 20/minute

inspiratory time 0.5 seconds

The infants spontaneous breathing rate is 40/minute.

The number of breaths per minute which are assisted by the ventilator is which one of the following?

A. 0.

B. 20.

C. 25.

D. 40.

E. 60.

QUESTION 82

A 13-year-old boy with focal segmental glomerulosclerosis presents to hospital with a four-day historyof vomiting and diarrhoea. He has not passed urine for at least 12 hours. Six weeks before, he had aserum creatinine of 0.11 mmol/L. He has been on 40 mg frusemide daily and an angiotensin-converting enzyme (ACE) inhibitor.

On examination, he is well-perfused, moderately oedematous, with a pulse of 110/minute and bloodpressure of 90/60 mmHg. His biochemistry on admission shows serum creatinine 0.50 mmol/L [0.05-0.08] and sodium 130 mmol/L [135-145]. His serum albumin was 12 g/L [35-50].

Which of the following would be the most appropriate next step in management?

A. Dialysis.

B. Fluid restriction.

C. Increase dose of frusemide.

D. Maintenance intravenous fluids.

E. Volume replacement with concentrated albumin solution.



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QUESTION 83

A six-year-old boy presents with a sore throat, high fever and anorexia. The appearance of his mouthand tongue is shown below.

The most likely organism responsible for this picture is:

A. Candida albicans.

B. Coxsackievirus A16.

C. Epstein-Barr virus.

D. Fusobacterium necrophorum.

E. herpes simplex virus.

QUESTION 84

A previously settled breastfed infant of four weeks demonstrates increasing crying behaviour over aweek. The infant continues to feed well, though fussily, and continues to gain weight. The babyeffortlessly vomits small amounts of milk four or five times a day. Faeces are watery, yellow and

occasionally explosive. The perianal skin is normal.

The change of behaviour is most likely to be associated with:

A. change in the mothers diet.

B. gastro-oesophageal reflux.

C. lactose intolerance.

D. normal variation in infant behaviour.

E. urinary tract infection.



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QUESTION 85

A six-year-old girl presents with a six-month history of staring spells associated with eye flickering andlip smacking movements. An electroencephalogram (EEG) is performed and is shown below.

Which of the following anticonvulsants is most appropriate?

A. Carbamazepine.

B. Clonazepam.

C. Phenytoin.

D. Sodium valproate.

E. Vigabatrin.



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QUESTION 86



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A nine-year-old boy presents to the emergency department with chest pain. He had cardiac surgery ininfancy, but the parents are vague about the diagnosis. His electrocardiogram (ECG) is shownopposite.

The most likely cardiac lesion was:

A. atrioventricular canal defect.

B. hypoplastic right ventricle.

C. perimembranous ventricular septal defect.

D. tetralogy of Fallot.

E. truncus arteriosus.

QUESTION 87

Which one of the following confers the greatest risk for the development of fungal infection in a patientundergoing chemotherapy?

A. Central venous lines.

B. Long-term antibiotics.

C. Prolonged neutropenia.

D. Use of monoclonal antibodies.

E. Use of steroids.

QUESTION 88

A 13-year-old girl presents with excessive bleeding following a tooth extraction. She has a history ofheavy menstrual loss. The following coagulation tests are obtained:

activated partial thromboplastin time (APTT) 40 seconds [27.5-34.5]

50:50 mix with normal plasma 32 seconds

prothrombin time (PT) 14 seconds [14-18]

thrombin clotting time (TCT) 16 seconds [14-18]

Which one of the following is the most likely diagnosis?

A. Dysfibrinogenaemia.

B. Factor XII deficiency.

C. Haemophilia A.

D. Lupus anticoagulant.

E. von Willebrand disease.



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QUESTION 89

A 12-year-old boy with longstanding asthma presents with a six-week history of increased cough anddyspnoea. The cough comes on suddenly in the day, but not usually at night. It lasts for a minute ortwo, during which time he finds it hard to get his breath. Usually his asthma is well controlled onregular betamethasone by inhaler two puffs twice daily, and he rarely needs to use his salbutamol

puffer. For the last month, however, he has often had to stop when playing soccer because of a boutof prolonged coughing.

The following results are obtained:


white cell count 18.6 x 109 /L [4.0-11.0]

neutrophils 2.5 x 109 /L [1.0-4.0]

lymphocytes 14.1 x 109 /L [3.0-7.0]

eosinophils 1.1 x 109 /L [


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QUESTION 91

A three-year-old boy presents with a 10-day history of cough and fever unresponsive to oralantibiotics. His chest X-ray is shown above.

Which of the following best explains the appearance shown on the X-ray?

A. Left-sided consolidation and pleural effusion.

B. Left-sided pulmonary abscess.

C. Obstruction of right main bronchus.

D. Right-sided pneumothorax.

E. Unilateral left-sided pulmonary oedema.



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QUESTION 92

A four-year-old girl is noted by her parents to have a funny looking eye (pictured). She has notcomplained of anything herself but seems to be bumping into things more often recently.

On examination, the only other finding is a swollen right knee. There is a history of back problems onher fathers side of the family. Her maternal grandmother has rheumatoid arthritis.

Which of the following tests is most likely to be diagnostically useful?

A. Anti-double stranded DNA antibody (anti-dsDNA).

B. Antinuclear antibody (ANA).

C. Antineutrophil cytoplasmic antibody (ANCA).

D. HLA-B27.

E. Rheumatoid factor.

QUESTION 93

A child presents with a sore throat. Which of the following clinical features is least likely in Group Astreptococcal tonsillitis?

A. Age 10 years.

B. Cough.

C. Exudate.

D. Fever >39C.

E. Tender enlarged cervical lymph nodes.



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QUESTION 94

A three-month-old boy is referred to clinic because an antenatal ultrasound showed a dilated left renalpelvis. This is confirmed by postnatal ultrasound. His left renal pelvic diameter is 25 mm [


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55 P204

QUESTION 95

A four-week-old girl presents with prolonged neonatal jaundice and poor weight gain. On examination,a small and firm liver is found and the spleen is palpable 2 cm below the left costal margin. She alsohas a harsh 3/6 systolic murmur radiating widely.

The following liver function tests were obtained:

total bilirubin 85 mol/L [0-15]

conjugated bilirubin 65 mol/L [0-5]

alanine aminotransferase (ALT) 45 U/L [


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56 P204

QUESTION 97

A five-month-old boy presents with chronic diarrhoea, failure to thrive and recent onset coughassociated with feeding intolerance. Blood test results show:


white cell count 14 x 109 /L [4-11]differential:

neutrophils 11 x 109 /L [1-7]

lymphocytes 3 x 109 /L [1-4]

platelet count 90 x 109 /L [150-400]

IgG 9.0 g/L [3.3-6.4]

IgA 0.67 g/L [0.30-0.70]

IgM 1.90 g/L [0.25-0.60]

A chest X-ray was taken and is shown below.

Which one of the following is the most likely cause of this clinical presentation?

A. Human immunodeficiency virus (HIV) infection.

B. Hyper IgM syndrome.

C. Severe combined immunodeficiency.

D. Wiskott-Aldrich syndrome.

E. X-linked agammaglobulinaemia.



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57 P204

QUESTION 98

A 10-year-old boy presents with a six-month history of lethargy and weight gain. History reveals thathe has been on thyroxine in the past and that his parents are first cousins. On examination he has afirm moderately sized goitre. His thyroid function tests are as follows:

free thyroxine (free T4) 9 pmol/L [10-20]thyroid-stimulating hormone (TSH) 37.00 mU/L [0.03-4.00]

Thyroid technetium pertechnetate scan is demonstrated below and shows increased uptake.

Which one of the following is the most likely diagnosis?

A. Autoimmune hypothyroidism.

B. Dyshormonogenesis.

C. Graves disease.

D. Iodine excess.

E. Thyroid dysgenesis.

QUESTION 99

It is considered inappropriate to perform predictive genetic testing on young children for Huntingtondisease, an adult-onset neurodegenerative disorder. Which of the following is the most importantreason for this?

A. Positive status for a Huntington disease mutation would block the childs future access to life,mortgage and income protection insurance.

B. Such testing is a violation of the childs autonomy, in that they lose the freedom to choosewhether to have the test later in life.

C. The main reason for testing adults for Huntington disease is to allow mutation carriers theoption of prenatal diagnosis, an issue which is irrelevant in childhood.

D. There is no currently available treatment for Huntington disease.

E. The testing process would place excessive psychological stress on a child, as children lackthe emotional maturity to cope with such testing.



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QUESTION 100

A multigravida is induced by artificial rupture of the membranes and syntocinon infusion at 41 weeksgestation. After 16 hours of labour she is noted to be fully dilated. Ninety minutes later, after fourunsuccessful attempts at delivery by vacuum extraction, she is taken to the operating theatre for anemergency Caesarean section. At delivery a 3800 g male infant is in poor condition requiring full

resuscitation. He is transferred, intubated and ventilated, to an intensive care unit. At 12 hours of agehe deteriorates with pallor and poor perfusion. His haemoglobin is 95 g/L and mean arterial bloodpressure is 30 mmHg. Extensive bruising is noted over his head. His head circumference is 38 cm.His pupils are unable to be examined due to marked oedema of his upper eyelids.

The most likely cause for his deterioration is blood loss due to:

A. cephalohaematoma.

B. intracerebral haemorrhage.

C. intraventricular haemorrhage.

D. subaponeurotic haemorrhage.

E. subdural haemorrhage.



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2004 FRACP Written Examination

Paediatrics & Child Health

Paper 2 Clinical Applications

Answers

1. D 34. B 67. B

2. E 35. B 68. C

3. E 36. A 69. A

4. C 37. A 70. B

5. B 38. D 71. A

6. D 39. D 72. D

7. E 40. C 73. D

8. D 41. A 74. A

9. A 42. A 75. C

10. E 43. D 76. C

11. A 44. A 77. E

12. C 45. A 78. C

13. E 46. C 79. B

14. E 47. B 80. E

15. B 48. E 81. D

16. B 49. C 82. E

17. A 50. B 83. E

18. A 51. D 84. D

19. D 52. A 85. D

20. A 53. B 86. A

21. D 54. E 87. C

22. C 55. A 88. E

23. D 56. B 89. D

24. C 57. A 90. C

25. A 58. D 91. A26. C 59. E 92. B

27. B 60. A 93. B

28. C 61. B 94. D

29. B 62. E 95. A

30. D 63. E 96. E

Documents

2004 FRACP Written Examination Paediatrics & Child