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2006 FRACP Written Examination

Paediatrics & Child Health

Paper 2 – Clinical Applications

INTRODUCTION

Format

Paper 1 – Medical Sciences: 70 questions; time allowed: 2 hours


Paper 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 options
given.

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 the
visual material.

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

Answers
A table of answers is located at the end of each paper.

Scoring
A correct answer will score one mark and an incorrect answer zero. There is no negative marking in
the FRACP Written Examination.

Queries
Contact the Examinations Section, Education Department via e-mail: exams@racp.edu.au.

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

Copyright © 2007 by The Royal Australasian College of Physicians

All Written Examination papers are copyright. They may not be reproduced in whole or part without
written permission from The Royal Australasian College of Physicians, 145 Macquarie Street, Sydney,
Australia.

Copyright © 2007 by The Royal Australasian College of Physicians


2 P206

QUESTION 1

A previously well 13-year-old boy presents for investigation of persistent lip swelling and bleeding
gums for the past four months. For about 12 months he has experienced colicky abdominal pains after
eating. He has had no vomiting or diarrhoea but complains of pain on defaecation, with some bright
red blood on the toilet paper. He has lost 3 kg in weight over the past six months. On examination he
looks pale and has marked gingivitis and swollen upper and lower lips. His abdomen is soft, without
palpable organomegaly, but with mild tenderness and fullness in the right iliac fossa. He has a perianal
skin tag.

What is the most likely diagnosis?

A. Chronic granulomatous disease.

B. Crohn's disease.

C. Gastrointestinal tuberculosis.

D. Sarcoidosis.

E. Systemic lupus erythematosus.

QUESTION 2

An eight-month-old girl starts to wake repeatedly at night after settling well to sleep. She is breastfed
to settle once she wakes. This has been occurring for three weeks despite her having slept through
from six-months of age. Her growth and development are normal. She eats a variety of solids and is
breastfed five to six times during the day.

Which of the following is the most likely explanation for her behaviour?

A. Diminishing breast milk production.

B. Food allergy.

C. Gastroesophageal reflux.

D. Inappropriate sleep association.

E. Nightmares.

QUESTION 3

When counselling parents of a child born with tetralogy of Fallot, you are asked what the risk is of
having an infant with any form of congenital heart disease in future pregnancies. The risk of
recurrence in this family is closest to:

A. 0.5%.

B. 2.5%.

C. 5%.

D. 12.5%.

E. 25%.

Copyright © 2007 by The Royal Australasian College of Physicians


3 P206

QUESTION 4

A 21-month-old girl presents, with her siblings, with a viral upper respiratory tract infection. Her facial
features are noted to be different from her siblings and this has been present since birth. A photograph
is shown (the patient is seated in the middle). She has no history of feeding or breathing difficulties as
an infant. On examination her eye movements are normal with normal pupillary responses. Her smile
is equal and symmetrical. What is the most likely diagnosis?

A. Bilateral facial nerve (VII) palsy.

B. Bilateral oculomotor (III) nerve palsy.

C. Congenital myasthenia gravis.

D. Congenital ptosis.

E. Möbius syndrome.

QUESTION 5

In the treatment of moderate depression in adolescents, which of the following has the best evidence
of efficacy?

A. Cognitive behavioural therapy.

B. Family therapy.

C. Fluoxetine.

D. Imipramine.

E. Interpersonal psychotherapy.

Copyright © 2007 by The Royal Australasian College of Physicians


4 P206

QUESTION 6

A four-year-old girl is seen in diabetes clinic. It is four months since she was diagnosed with type 1
(insulin-dependent) diabetes mellitus and coeliac disease. Her parents report marked behavioural
problems and temper tantrums, associated with diabetes treatment and dietary restrictions.

On examination extensive hairloss is noted as shown in the photograph above.

What is the most likely diagnosis?

A. Alopecia areata.

B. Telogen effluvium.

C. Tinea capitis.

D. Traction alopecia.

E. Trichotillomania.

QUESTION 7

A 13-month-old male infant is admitted to hospital with acute gastroenteritis and dehydration.
Investigations included full blood count, electrolytes and liver function tests. All are normal apart from a
raised alkaline phosphatase of 1350 U/L [30-300]. An X-ray of the knee was normal. The most likely
diagnosis is:

A. Alagille syndrome.

B. cystic fibrosis.

C. osteogenesis imperfecta.

D. transient hyperphosphatasaemia.

E. vitamin D resistant rickets.

Copyright © 2007 by The Royal Australasian College of Physicians


5 P206

QUESTION 8

A mutation at the 5' end of an intron may interfere with splicing and be pathogenic. Which statement
best summarises the consequence of such a mutation?

A. The abnormal intron blocks transcription.

B. The abnormal intron interferes with post-translational processing.

C. The abnormal intron is included in the mRNA strand.

D. The adjacent exon is duplicated.

E. The adjacent exon is not included in the mRNA strand.

QUESTION 9

A full-term neonate is born following a pregnancy complicated by polyhydramnios. The baby was
hydropic, and died at six hours of age.

Which one of the following is least likely to have caused this?

A. Alpha-thalassaemia.

B. Beta-thalassaemia.

C. Blackfan-Diamond anaemia.

D. Fetal parvovirus B19 infection.

E. Rh disease of the newborn.

QUESTION 10

The diagnosis of neurofibromatosis type 1 (NF-1) is made in a seven-year-old boy. He is


developmentally normal. Of the following, which complication of NF-1 is he most likely to develop
during childhood or adolescence?

A. Hypertension.

B. Malignant transformation of a neurofibroma.

C. Scoliosis.

D. Seizures.

E. Sensorineural deafness.

Copyright © 2007 by The Royal Australasian College of Physicians


6 P206

QUESTION 11

A clinically well child with the dysmorphic features shown in the photograph below presents with a loud
ejection systolic murmur.

The child undergoes cardiac catheterisation which yields the following information:

Saturation Pressure (mmHg)


superior vena cava 64%
right atrium 62% 9/6 (mean 8)
right ventricle 62% 51/7
main pulmonary artery 62% 51/15 (mean 35)
distal left pulmonary artery 62% 12/9 (mean 11)
distal right pulmonary artery 62% 14/9 (mean 12)
left ventricle 98% 66/8
descending aorta 98% 70/40 (mean 53)

Which of the following is the most likely diagnosis?

A. Alagille syndrome.

B. Congenital rubella syndrome.

C. Noonan syndrome.

D. Velocardiofacial syndrome.

E. Williams syndrome.

Copyright © 2007 by The Royal Australasian College of Physicians


7 P206

QUESTION 12

A six-year-old Sudanese male presents to emergency appearing toxic with a temperature of 39.4oC.
He has a three-day history of fevers and a 24-hour history of left elbow pain and swelling. Blood tests
at this time are as follows:

haemoglobin 70 g/L [115-155]


white cell count 18.9 x 109/L [4.5-14.5]
absolute neutrophil count 10500 [1500-8000]
platelets 530 x 109/L [150-400]
reticulocytes 15.5% [<2%]
C-reactive protein (CRP) 90 mg/L [0-10]

A photomicrograph of the blood film is shown above. Which of the following organisms is most likely
to cause this clinical presentation?

A. Escherichia coli.

B. Haemophilus influenzae.

C. Salmonella enteritidis.

D. Staphylococcus epidermidis.

E. Yersinia enterocolitica.

Copyright © 2007 by The Royal Australasian College of Physicians


8 P206

QUESTION 13

A 12-year-old boy with a past history of allogeneic bone marrow transplantation (BMT) for severe
aplastic anaemia is on cyclosporin and prednisone for active extensive chronic graft-vs-host disease
(GVHD). He presents to emergency with a high fever. The following investigations were performed:

Haemoglobin (Hb) 129 g/L [125-170]


White cell count (WCC) 26.5 x109/L [4.5-13]
Platelets 91 x109/L [150-400]
Sodium 136 mmol/L [37-145]
Potassium 4.2 mmol/L [3.5-5]
HCO2 18 mmol/L [22-30]
Urea 10.8 mmol/L [3.2-7.1]
Creatinine 0.221 mmol/L [<0.133]
C-reactive protein (CRP) 133 mg/L [0 -10]

His blood film shows Howell-Jolly bodies. Which one of the following organisms is most likely
responsible for his clinical presentation?

A. Escherichia coli.

B. Pseudomonas aeruginosa.

C. Staphylococcus aureus.

D. Streptococcus pneumoniae.

E. Streptococcus viridans.

QUESTION 14

A previously well two-year-old boy accidentally ingests a large dose of ibuprofen, estimated to be 6-8
times the recommended dose. Emergency decontamination of his stomach is carried out in the
Emergency department. Two hours after ingestion, his serum creatinine is 0.05mmol/l [0.03-0.07].

What is the next most appropriate next step?

A. Check serum creatinine in 6-8 hours.

B. Forced alkaline diuresis.

C. High dose prednisolone.

D. Prostaglandin agonist.

E. Urgent urinalysis.

Copyright © 2007 by The Royal Australasian College of Physicians


9 P206

QUESTION 15

In the pedigree shown below, the individual indicated by a solid symbol is affected by an autosomal
recessive disorder. No other family members are affected.

Which of the marked individuals is least likely to be a carrier of this disorder?

C D

A. A.

B. B.

C. C.

D. D.

E. E

QUESTION 16

Neuroadaptation (tolerance) syndrome is the phenomenon of loss of treatment response generally


after months of a clear and definite response to medication. With which of the following medications is
this most likely to occur in children?

A. Carbamazepine.

B. Clonazepam.

C. Fluoxetine.

D. Methylphenidate.

E. Sodium valproate.

Copyright © 2007 by The Royal Australasian College of Physicians


10 P206

QUESTION 17

A two-year-old boy presents with a history of constipation for several months and recent ataxia. He is
otherwise well. On examination he is ataxic without other neurological signs. He has a non-tender
(right) upper abdominal mass. Computed tomography (CT) scans of his head and abdomen are shown
below.

Which one of the following diagnoses is most likely?

A. Alveolar rhabdomyosarcoma.

B. Desmoplastic round cell tumour of the abdomen.

C. Ewing sarcoma.

D. Neuroblastoma.

E. Wilms tumour.

Copyright © 2007 by The Royal Australasian College of Physicians


11 P206

QUESTION 18

An otherwise normal six-year-old boy is diagnosed with moderate sensorineural hearing loss. There is
no significant family history.

The most likely cause of his hearing loss is:

A. congenital cytomegalovirus.

B. congenital rubella.

C. Connexin 26 mutation.

D. Pendred syndrome.

E. Usher syndrome.

QUESTION 19

A five-year-old boy is referred to the haematology clinic with a history of easy bruising and excessive
bleeding from mild trauma.

The following investigations were undertaken:

Haemoglobin (Hb) 109 g/L [115-155]


White cell count (WCC) 7.7x109/L [4.5-14.5]
Platelets 257x109/L [150-400]
Prothombin time 13.7 second [14-18]
Activated partial thromboplastin time 72.1 seconds [22.5-34.5]
50:50 Mix 30.4 seconds
Thrombin clotting time 15.5 seconds [14-18]
F.VIIIc 3% [50 -150]
vWF Ristocetin cofactor 18% [50-200]
vWF Antigen 21% [50-200]
Collagen Binding Assay 14% [50-200]

The most likely diagnosis is:

A. Factor VII deficiency.

B. Glanzmann’s syndrome.

C. Haemophilia A.

D. Haemophilia B.

E. von Willebrand Disease.

Copyright © 2007 by The Royal Australasian College of Physicians


12 P206

QUESTION 20

A seven-year-old boy presents with moderate proteinuria and macroscopic haematuria associated with
a sore throat and fever. His renal function, C3, C4 and renal ultrasound are normal. Microscopic
haematuria persists and he continues to have intermittent macroscopic haematuria with intercurrent
infections. His mother also has microscopic haematuria. His maternal grandfather died of renal
failure.

The electron microscopy of his renal biopsy shows variable thickness of the glomerular basement
membrane and is shown below.

The most likely diagnosis in this boy is:

A. Alport syndrome.

B. benign familial haematuria.

C. IgA nephropathy.

D. mesangiocapillary glomerulonephritis.

E. post-infectious glomerulonephritis.

Copyright © 2007 by The Royal Australasian College of Physicians


13 P206

QUESTION 21

A four-year-old boy is brought to the emergency department with a one day history of fever, vomiting
and lethargy. His parents are concerned that he is pale and that his eyes look yellow. On examination
his temperature is 37.5°C, heart rate is 110/minute, blood pressure 90/60 mmHg and respiratory rate
is 25/minute. He is pale and quiet, but responsive, with mildly icteric sclera. His examination is
otherwise unremarkable with no evidence of hepatosplenomegaly. A urinalysis is performed and is
positive for blood. His blood test results are displayed below.

Haemoglobin (g/L) 78 [116-136]


MCV (fL) 83 [75-85]
MCH (pg) 29 [24-30]
MCHC (g/L) 345 [310-360]
9
Platelets (x 10 /L) 292 [150-450]
Reticulocytes (x 109/L) 154.2 [20-105 x 109/L]
Heinz Bodies Negative
Poikilocytosis +
Spherocytosis Occasional
White Blood Cells (x 109/L) 5.83 [6.10-11.00]
Bilirubin Total (µmol/L) 84 [2-20]
Bilirubin Conjugated (µmol/L) 8 [<10]
Haptoglobin (g/L) < 0.06 [0.35-2.10]
Direct Antiglobulin Test Poly Negative
Direct Antiglobulin Test IgG Negative
Direct Antiglobulin Test C3d Negative

Which of the following diagnoses is most likely?

A. Cold antibody autoimmune haemolytic anaemia.

B. Glucose-6-phosphate dehydrogenase deficiency.

C. Hereditary spherocytosis.

D. Paroxysmal nocturnal haemoglobinuria.

E. Warm antibody autoimmune haemolytic anaemia.

QUESTION 22

A ten-year-old boy presents after a hypoglycaemic fit preceded by 24 hours of vomiting. He has a past
medical history of asthma with no recent exacerbations or significant interval symptoms. He is on a
Seretide Accuhaler (500 micrograms of fluticasone and 50 micrograms salmeterol), one inhalation
twice daily. His physical examination is unremarkable.

Which investigation is most likely to confirm the cause of his hypoglycaemia?

A. Adrenal antibodies.

B. Adrenocorticotrophic Hormone (ACTH) level.

C. Random cortisol level.

D. Stimulated cortisol level.

E. Very long chain fatty acid levels.

Copyright © 2007 by The Royal Australasian College of Physicians


14 P206

QUESTION 23

A previously well eight-year-old boy presents to the emergency department with two days of persistent
vomiting and several loose bowel motions. He has had no urine output for at least 12 hours.
Examination shows that he is mildly dehydrated but his vital signs are normal for age. He is given a
bolus of 500mL of 0.9% saline intravenously and discharged home with advice to visit the family
doctor the next day. He represents five hours later with persistent vomiting and increasing lethargy. He
passes 20ml of dark urine.

Initial tests show the following:

Sodium 132 mmol/L [134-144]


Potassium 7.5 mmol/L [3.5-5.0]
Urea 39 mmol/L [3.0-7.5]
Creatinine 0.35 mmol/L [0.04-0.08]
Haemogloblin 105 g/L [120-155]
9
Platelet count 235 x 10 [150-400]

Urinalysis shows 3+blood, 3+protein

The most likely diagnosis is:

A. acute dehydration.

B. acute glomerulonephritis.

C. acute interstitial nephritis.

D. acute tubular necrosis.

E. nephrotic syndrome.

QUESTION 24

A ten-year-old boy is investigated for excessive day time sleepiness. He sleeps 11 hours per night and
snores most nights. He wakes frequently at night and calls out to his mother.

He describes periods of being not being able to move in bed as though he is paralysed. He
occasionally falls to the ground when laughing.

The most likely diagnosis is:

A. atonic epilepsy.

B. narcolepsy.

C. nocturnal epilepsy.

D. obstructive sleep apnoea.

E. parasomnia.

Copyright © 2007 by The Royal Australasian College of Physicians


15 P206

QUESTION 25

A three-and-a-half-year-old boy is referred because his parents are concerned about his poor social
skills. He prefers to be alone. He can be quite insistent on routines and is easily upset if he does not
get his way. Some of his tantrums last up to half an hour. He is interested in mechanical things such
as DVD, tape recorder etc. His attentional skills are not good, however he has recently developed
some good imaginative play. He has good expressive and receptive language skills. He has no
repetitive behaviours or unusual body movement and no fascinations or obsessions.

He is an only child. His father tends to be obsessive and anxious, and his mother was very shy when
she was small. A first cousin has autism.

He is very wary of you initially, but after some time begins to respond to your requests and follows
some single and two stages commands. He is quiet with poor eye contact, but has some reasonably
communicative facial expressions and gestures. A single five word sentence is heard. Physically
examination is normal. His weight and height are both on the 10th percentile for age, head
circumference 50th percentile. Before leaving he brings a toy car out of his pocket to show you.

What is the most likely diagnosis?

A. Asperger syndrome.

B. Attention deficit hyperactivity disorder.

C. Autism.

D. Fragile X syndrome.

E. Normal child.

QUESTION 26

A ten-year-old boy presents with facial swelling, hypertension, proteinuria and haematuria.
Complement levels are 0.7 mg/dL [0.8-1.8]; antistreptolysin-O titre (ASOT) and anti-DNase are
positive. He is excreting 500 mg of urinary protein in 24 hours [<200]. Anti-nuclear antibodies are
negative, and he has no other symptoms or signs suggesting autoimmune phenomena. After initial
treatment with fluid restriction, diet and antihypertensives, his urinary sediment improves, and blood
pressure returns to normal. At two months he is reassessed.

Which of the following would most strongly indicate the need for renal biopsy?

A. Creatinine of 0.10 mmol/L [0.05 – 0.10].

B. Haematuria.

C. Hypertension.

D. Hypocomplementaemia.

E. Proteinuria of > 200 mg/24 hours.

Copyright © 2007 by The Royal Australasian College of Physicians


16 P206

QUESTION 27

A four-year-old girl presents with a two to three day history of increasing lethargy and pallor following a
recent febrile illness associated with cough. Parents report her urine appeared dark that morning. On
examination she is clinically jaundiced and tachycardic but well perfused and has mild splenomegaly.

The following investigations were obtained:

Haemoglobin (Hb) 67 g/L [110-145]


Mean corpuscular volume 90 fL [72-87]
White cell count (WCC) 11.5 x 109/L [5-17]
Platelets 426 x 109/L [150-400]
Reticulocytes 10%
Bilirubin 157µmol/L [0-20]
Aspartate aminotransferase (AST) 141U/L [0-35]
Lactate Dehydrogenase (LDH) 11,751 U/L [500-920]
Creatinine 0.032 mmol/L [<0.062]

A photomicrograph of the blood film is shown above. Which is the most appropriate next step in her
management?

A. Haemodialyse.

B. Intravenous gammaglobulin.

C. Intravenous methylprednisolone.

D. Transfuse packed red cells.

E. Warm the child.

Copyright © 2007 by The Royal Australasian College of Physicians


17 P206

QUESTION 28

A nine-month-old girl is referred by her general practitioner because of recurrent events occurring on a
daily basis over the last two weeks. The episodes are stereotyped and consist of her stopping what
she is doing, flexing at her trunk, and pressing her hands above her inguinal region. There is
associated tremulousness and jaw rigidity. The events last one to two minutes with her becoming red
in the face and grunting. She seems to be preoccupied and gets distressed if touched or moved.
After the event, the child goes to sleep. The events never occur in sleep.

The most likely diagnosis is:

A. dystonia.

B. frontal lobe seizures.

C. gastroesophageal reflux.

D. infantile masturbation.

E. urinary infection.

QUESTION 29

A four-year-old boy is reviewed in clinic because of a flare-up of his atopic eczema. He has not
responded to his usual emollients and topical steroids.

On examination his temperature is 37°C. He is irritable, and has extensive whole-body eczema with
excoriation and crusting. The lesions around his mouth are shown above.

The most appropriate treatment is:

A. aciclovir.

B. flucloxacillin.

C. more potent topical steroid.

D. mupirocin ointment.

E. pimecrolimus cream.

Copyright © 2007 by The Royal Australasian College of Physicians


18 P206

QUESTION 30

Following curative therapy, which one of the following primary malignancies is most likely associated
with a future second malignant neoplasm?

A. Acute lymphoblastic leukaemia.

B. Hodgkin lymphoma.

C. Medulloblastoma.

D. Sporadic retinoblastoma.

E. Wilms tumour.

QUESTION 31

An eight-year-old boy is referred to you with tiredness and short stature. He has a long history of thirst
and nocturia. There is no family history of note. On examination he is short compared with his five-
year-old sister. Apart from obvious pallor, there are no other abnormal physical features.

Investigations show the following:


mid-stream urine 50 leucocytes
20 red cells
trace protein
no bacterial growth
haemogloblin 74 g/L [115-155]
serum creatinine 0.20 mmol/L [0.03-0.07]

What is the most likely diagnosis?

A. Familial juvenile nephronophthisis.

B. Medullary cystic disease.

C. Polycystic kidney disease.

D. Reflux nephropathy.

E. Renal Fanconi syndrome.

QUESTION 32

Which one of the following medications is most likely to cause foetal abnormalities if taken in the first
trimester of pregnancy?

A. Enalapril.

B. Gentamicin.

C. Ibuprofen.

D. Tetracycline.

E. Warfarin.

Copyright © 2007 by The Royal Australasian College of Physicians


19 P206

QUESTION 33

A nine-year-old girl with systemic lupus erythematosus (SLE) presents with a painless, erythematous,
blistering rash on her chest, as shown. She is afebrile. She is currently on prednisone, and has
recently been given pulse cyclophosphamide.

The most appropriate initial treatment would be:

A. intravenous aciclovir.

B. intravenous flucloxacillin.

C. oral aciclovir.

D. oral flucloxacillin.

E. withhold immunosuppressants.

QUESTION 34

In children with reading disability (developmental dyslexia) the underlying neurocognitive deficit most
commonly occurs in:

A. attention.

B. auditory processing.

C. phonemic awareness.

D. saccadic visual tracking.

E. sensorimotor perception.

Copyright © 2007 by The Royal Australasian College of Physicians


20 P206

QUESTION 35

A nine-month-old child is reviewed because of concerns about the appearance of his face. He
suffered birth trauma related to shoulder dystocia. A brachial plexus injury was identified at birth.

What level of the brachial plexus has been injured to explain the facial findings?

A. C5.

B. C6.

C. C7.

D. C8.

E. T1.

QUESTION 36

What is the prefered pharmacological treatment for attention deficit hyperactivity disorder in children
with comorbid intellectual disability?

A. Clonidine.

B. Fluoxetine.

C. Haloperidol.

D. Methylplenidate.

E. Risperidone.

Copyright © 2007 by The Royal Australasian College of Physicians


21 P206

QUESTION 37

A two-year-old child is referred for assessment of a murmur. Examination reveals a mid-diastolic


murmur without a systolic component. No systolic murmur is audible. There is associated
hepatomegaly and distension of neck veins, but no respiratory distress. The electrocardiogram (ECG)
is shown below.

The most likely diagnosis is:

A. aortic valve regurgitation.

B. cortriatriatum.

C. mitral valve stenosis.

D. pulmonary valve regurgitation.

E. tricuspid valve stenosis.

QUESTION 38

A seven-year-old girl who underwent surgical repair of coarctation of the aorta as a neonate requires a
dental extraction because of severe dental caries. She has no known drug allergies. The most
appropriate recommendation for antibiotic prophylaxis is:

A. intravenous amoxycillin and intravenous gentamicin 30 minutes before and six hours after
procedure.

B. no antibiotic prophylaxis is required.

C. oral amoxycillin one hour before procedure.

D. oral azithromycin one hour before procedure.

E. oral clindamycin one hour before and six hours after procedure.

Copyright © 2007 by The Royal Australasian College of Physicians


22 P206

QUESTION 39

A 15-year-old male has recurrent haematuria. He had microscopic haematuria noted during an
admission with pneumonia six months earlier. Follow up urine microscopy was normal a month later.
He has developed macroscopic haematuria following a recent throat infection which lasted six days.
Examination is unremarkable.

Investigations show a normal full blood count, creatinine, electrolytes, calcium, liver function, clotting
screen and chest radiograph. A mid-stream specimen of urine shows 90 red blood cells per high
powered field but no casts or protein. Urine culture is sterile.

The most likely diagnosis is:

A. Alport.

B. Haemohorregic cystitis.

C. Henoch-Schönlein purpura.

D. IgA nephropathy.

E. Wegener’s granulomatosis.

QUESTION 40

A 15-month-old infant has itchy lesions on the scalp, neck, palms and soles (as shown above). The
most likely diagnosis is:

A. Coxsackie A16 infection (Hand, foot and mouth disease).

B. eczema herpeticum.

C. impetigo.

D. pompholyx (dyshidrotic eczema).

E. scabies.

Copyright © 2007 by The Royal Australasian College of Physicians


23 P206

QUESTION 41

A 12-year-old boy presents with a two year history of tremor predominantly affecting his hands. The
tremor is worse when he is anxious, upset or active. There is a family history of tremor in a paternal
grandparent.

On examination, the tremor is present when his hands are outstretched in front of him. The tremor
worsens when asked to perform finger-to-nose movements. Examination is otherwise normal,
including gait.

The most likely diagnosis is:

A. anxiety.

B. cerebellar tremor.

C. dystonia.

D. essential tremor.

E. Wilson disease.

QUESTION 42

A 15-year-old boy presents to the emergency department with a brief history of being agitated and
confused. He is febrile, sweating and tachycardic, and has dilated pupils. He has increased limb tone,
hyperreflexia, and myoclonic jerking of limbs.

He has a long history of behaviour disturbance, and has been in foster care for several months as he
was unable to be contained at home. His carers report that he has been prescribed the selective
serotonin reuptake inhibitor paroxetine, but they are unsure whether he has been taking it. He has
been out late in the evenings recently.

What is the most likely diagnosis?

A. Inhalent abuse (chroming).

B. Marijuana-induced psychosis.

C. Methylenedioxymethamphetamine (MDMA, “ecstasy”) overdose.

D. Neuroleptic malignant syndrome.

E. Serotonin syndrome.

Copyright © 2007 by The Royal Australasian College of Physicians


24 P206

QUESTION 43

A newborn infant is noted to be tachypnoeic in the post natal ward. His chest X-ray is shown below.

What is the most likely diagnosis?

A. Congenital cystic adenomatoid malformation.

B. Congenital diaphragmatic hernia.

C. Congenital lobar emphysema.

D. Intralobar sequestration.

E. Pulmonary interstitial emphysema.

Copyright © 2007 by The Royal Australasian College of Physicians


25 P206

QUESTION 44

An eight-year-old boy presents with an isolated rash on his foot as shown above. The most
appropriate initial management is:

A. oral griseofulvin.

B. oral prednisolone.

C. oral terbinafine.

D. topical mometasone.

E. topical terbinafine.

QUESTION 45

With careful dose-titration and monitoring, short-term treatment with methylphenidate has been shown
to be efficacious for the treatment of attention deficit hyperactivity disorder (ADHD). If this treatment for
children with ADHD is maintained for the long-term, which of the following combinations best
represents the ongoing efficacy and impact upon weight and height?

Efficacy Weight Height


A. - - -
B. - ↓ -
C. - ↓ ↓
D. ↓ ↓ -
E. ↓ ↓ ↓

KEY: - = maintain ↓ = reduction

Copyright © 2007 by The Royal Australasian College of Physicians


26 P206

QUESTION 46

A ten-week-old male infant presents to the emergency department with a two day history of coryza
and poor feeding. He has not had a wet nappy for at least eight hours. His older brother has had an
upper respiratory tract infection over the past week. On examination, he is tachypnoeic with respiratory
rate of 80/minute, pulse rate 152/minute and is mildly febrile. His oxygen saturation on monitoring was
92% in air and auscultation of his chest revealed bilateral crackles.

In addition to supplemental oxygen, which of the following should be commenced?

A. Intravenous antibiotics.

B. Intravenous dexamethasone.

C. Intravenous fluids.

D. Nasal CPAP (Continuous Positive Airway Pressure).

E. Nebulized salbutamol.

QUESTION 47

A 15-year-old girl, the eldest of four children whose parents have separated, is referred to the
emergency department because of aggressive behaviour. Over the past week the girl has hit three of
her siblings and been verbally abusive to her mother. At school she has been distracted, irritable and
unable to settle, but has not previously been known as a trouble-maker. She refuses to be interviewed,
loudly swears at a nurse and lifts her skirt at the parents of two other patients. Physical examination is
difficult as she is uncooperative, but there are no abnormal findings.

The diagnosis that best fits this clinical picture is:

A. major depressive disorder.

B. manic episode.

C. oppositional defiant disorder.

D. physical abuse.

E. post-traumatic stress disorder.

QUESTION 48

A ten-year-old boy has nephrogenic diabetes insipidus. He consumes a normal diet and is well. His
daily urine output would be most effectively controlled by reducing which one of the following food
items?

A. Carbohydrate.

B. Fat.

C. Potassium.

D. Protein.

E. Sodium.

Copyright © 2007 by The Royal Australasian College of Physicians


27 P206

QUESTION 49

In patients who have previously received radiotherapy, exposure to which one of the following
chemotherapy agents is most likely responsible for the skin reaction as shown?

A. Cisplatin.

B. Cyclophosphamide.

C. Daunorubicin.

D. Etoposide.

E. Methotrexate.

QUESTION 50

A four-year-old boy presents with a long history of recurrent febrile episodes associated with mouth
ulcers, diarrhoea, fatigue and bleeding gums. The most helpful test to determine the diagnosis is:

A. neutrophil chemotaxis studies.

B. neutrophil count between episodes.

C. neutrophil count during an episode.

D. neutrophil respiratory burst studies.

E. twice weekly neutrophil counts for six weeks.

Copyright © 2007 by The Royal Australasian College of Physicians


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QUESTION 51

A three-year-old boy presents with episodes of loss of consciousness related to exercise and also
minor trauma. During the episodes he becomes pale, his eyes may roll upwards and he has had
urinary and faecal incontinence. His electrocardiogram (ECG) is shown above. What is the most
likely diagnosis?

A. Aortic stenosis.

B. Breath holding episodes.

C. Long Q -T syndrome.

D. Primary pulmonary hypertension.

E. Seizure disorder.

Copyright © 2007 by The Royal Australasian College of Physicians


29 P206

QUESTION 52

The audiogram of a six-year-old boy who completed chemotherapy for hepatoblastoma four weeks
ago is shown below.

The audiogram is most consistent with:

A. conductive hearing loss.

B. high-frequency sensorineural hearing loss.

C. low-frequency sensorineural hearing loss.

D. non-compliance with testing.

E. normal findings for age.

Copyright © 2007 by The Royal Australasian College of Physicians


30 P206

QUESTION 53

An infant weighing 10kg presents with fever and a rash as shown below. His heart rate is 150/minute
and blood pressure is 85/35 mmHg. Which of the following is the most appropriate initial intravenous
fluid management?

A. 20 mL/hour of 0.9% saline.

B. 40 mL/hour of 0.18% saline with 4% glucose.

C. 40 mL/hour of 0.9% saline.

D. 200 mL bolus of 0.18% saline with 4% glucose.

E. 200mL bolus of 0.9% saline.

QUESTION 54

In the treatment of patients with anorexia nervosa, the administration of nocturnal nasogastric feeds to
supplement a normal meal plan has been shown to:

A. increase long-term weight gain.

B. increase short-term weight gain.

C. make no difference to weight gain.

D. reduce long-term weight gain.

E. reduce short-term weight gain.

Copyright © 2007 by The Royal Australasian College of Physicians


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QUESTION 55

You are asked to see a term 4.1kg newborn infant who is now six hours old. The nursing staff in the
special care nursery are concerned that the infant may be having seizures. In two separate episodes,
the infant had jerking of the upper limbs that lasted for two to three minutes.

The infant was admitted to the nursery after a vaginal delivery with a prolonged second stage of
labour. Blood gas analysis from the umbilical cord at the time of delivery revealed pH 7.03 [7.34-7.43],
pO2 18 mmHg [50-90], pCO2 90 mmHg [31-42], base deficit of -24 [Base excess -5-+5].

You arrive to find a male infant who has eyes tonically deviated to the left, and has 30 seconds of
rhythmic jerking of the left upper limb. The infant then cries briefly before settling. Your initial
investigations are as follows:

Sodium 134 mmol/l [135-145]


Potassium 5.3 mmol/l [3.7 - 6.0]
Calcium 1.8 mmol/l [1.90 - 2.70]
Glucose 2.1 mmol/l [3.0 - 8.0]
Creatinine 0.08 mmol/L [0.01 - 0.03]
International normalised ratio (INR) 1.7 [0.8 - 1.5]
Haemoglobin (Hb) 176 g/L [145 - 225]
9
White cell count (WCC) 17.0 x 10 /L [5.0 - 25.0]
9
Platelets 172 x 10 /L [150 - 400]

The most likely cause of this infant's seizures is:

A. hypocalcaemia.

B. hypoglycaemia.

C. hypoxic-ischaemic encephalopathy.

D. intracranial haemorrhage.

E. stroke.

QUESTION 56

A child with egg allergy is most likely to develop an adverse reaction to which of the following
vaccines?

A. Influenza.

B. Measles-mumps-rubella.

C. Pneumococcal.

D. Polio.

E. Varicella.

Copyright © 2007 by The Royal Australasian College of Physicians


32 P206

QUESTION 57

A seven-month-old girl is referred to outpatient clinic for assessment of a misshapen head. She
weighs 7.4 kg (50th percentile), measures 66.5 cm (50th percentile) and her head circumference is 40
cm (5th percentile). Apart from her head shape there are no dysmorphic features. She does not roll,
does not reach and has slight head lag on pull to sit.

Her head and facial appearance is shown below.

The most likely reason for this appearance is:

A. coronal craniosynostosis.

B. deformational plagiocephaly.

C. lambdoid craniosynostosis.

D. metopic craniosynostosis.

E. sagittal craniosynostosis.

Copyright © 2007 by The Royal Australasian College of Physicians


33 P206

QUESTION 58

A four-year-old boy with known peanut allergy and asthma developed angio-oedema of the lips
followed by generalised urticaria within minutes of blowing up a balloon at a birthday party. An
ambulance was called and on arrival he was wheezy and distressed. Intra-muscular adrenalin was
given with total resolution of symptons within 20 minutes. The most likely cause of his reaction was:

A. biphasic reaction to inadvertant peanut exposure.

B. IgE mediated reaction to inadvertant peanut exposure.

C. IgE mediated reaction to latex exposure.

D. peanut induced exacerbation of asthma.

E. T cell mediated reaction to latex exposure.

QUESTION 59

None of the seven children of a profoundly deaf couple has any hearing impairment (see pedigree
below). The most likely explanation for this is:

A. chance.

B. one or both of the parents has an autosomal dominant form of deafness, which is highly
variable in its expression.

C. one or both of the parents has a non-genetic form of deafness.

D. the father has X-linked deafness and the mother has autosomal recessive deafness.

E. the parents have autosomal recessive deafness due to mutations in different genes.

Copyright © 2007 by The Royal Australasian College of Physicians


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QUESTION 60

A 13-year-old girl presents with a fractured ankle requiring surgical fixation.

Full blood examination shows:

haemoglobin 105 g/L [120-160]


red cell count 5.6 x 1012/L [4.0-5.7]
mean cell volume 62 fL [80-97]
white cell count 12.5 x 109/L [4.0-11.0]
platelet count 390 x 109/L [150-400]

Haemoglobin (Hb) studies:

HbA2 5.2% [1.8-3.5]


HbF 1.2% [0-2.0]
HbH preparation no HbH bodies seen
Hb electrophoresis no abnormal Hb bands seen

The most likely explanation for her anaemia is:

A. α-thalassaemia trait.

B. β-thalassaemia trait.

C. congenital sideroblastosis.

D. iron-deficiency anaemia.

E. sickle cell trait.

QUESTION 61

A 13-year-old girl who has a body mass index (BMI) of 31kg/m2 [95th centile for age and sex =
28kg/m2], is investigated for obesity. Her periods are irregular and she has a family history of
diabetes. On examination she has acanthosis nigricans of the neck and the axillae. Investigations are
shown.

Alanine aminotransferase (ALT) 89 U/L [0-30]


Aspartate aminotransferase (AST) 84 U/L [10-30]
Bilirubin 15 umol/L [<20]

The most likely cause of these findings is:

A. gallstones.

B. non-alcoholic fatty liver disease.

C. polycystic ovary disease.

D. type 2 diabetes mellitus.

E. viral hepatitis.

Copyright © 2007 by The Royal Australasian College of Physicians


35 P206

QUESTION 62

A two-year-old boy is admitted with fever of 39°C and refusal to walk. On examination he looks
lethargic and unwell. His left leg is painful and hot to touch.

Photographs of his leg and tongue are shown below.

The best empiric antibiotic for a child with this clinical picture is:

A. amoxycillin-clavulanate.

B. cefotaxime.

C. flucloxacillin.

D. penicillin.

E. trimethoprim-sulphamethoxazole.

QUESTION 63

A two-month-old female infant presents with a three week history of a scaly erythematous patchy rash
on the face and arms. Her mother is known to have systemic lupus erythematosis but is in remission.
The auto-antibody most likely to be positive on testing is:

A. anti-double-stranded DNA.

B. anti-Jo-1.

C. anti-Ro/SS-A.

D. anti-RNP.

E. anti-Sd-70.

Copyright © 2007 by The Royal Australasian College of Physicians


36 P206

QUESTION 64

In the context of IgE mediated food allergy, skin prick testing is likely to predict which one of the
following on subsequent exposure to the positive food?

A. Duration of food allergy.

B. Duration of symptoms.

C. Likelihood of a reaction (independent of severity).

D. Severity of a reaction.

E. Timing (onset) of symptoms.

QUESTION 65

A 16-year-old male has a past history of Hodgkin disease treated with chemotherapy. He presents
with a chronic dry cough. His chest X-ray is shown below.

Which of the following drugs is most likely responsible for the radiographic changes?

A. Bleomycin.

B. Busulphan.

C. Carmustine.

D. Cyclophosphamide.

E. Methotrexate.
.

Copyright © 2007 by The Royal Australasian College of Physicians


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QUESTION 66

A 15-year-old male with Duchenne muscular dystrophy is referred for evaluation of daytime tiredness,
poor school progress and headaches on rising in the morning. On examination he has a weak cough,
and air entry is reduced in both lung bases.

Spirometry reveals the following:

Forced Vital Capacity (FVC) 60% predicted


Forced Expiratory Volume in one second (FEV1) 55% predicted
Maximal mid expiratory flow (MMEF); 55% predicted.

An overnight polysomnogram demonstrates an Obstructive Respiratory Disturbance Index of


3.5/Hr [< 1].

A blood gas examination demonstrates the following:

pH 7.34 [7.34-7.45]
pCO2 59mmHg [34-45]
HCO3 35mmHg [20-28]

What is most appropriate treatment?

A. Adenotonsillectomy.

B. Bilevel ventilation (Bilevel positive airways pressure).

C. Continuous positive airway pressure treatment (CPAP).

D. Supplemental oxygen therapy.

E. Ventilation via tracheostomy.

QUESTION 67

The mother of a four-week-old boy is concerned that her infant has small streaks of bright red blood
and mucus in his stools. The infant is entirely breast-fed and otherwise well. The physical examination
is unremarkable.

What is the most likely diagnosis?

A. Allergic proctocolitis.

B. Anal fissure.

C. Duplication cyst.

D. Gastroenteritis.

E. Rectal polyp.

Copyright © 2007 by The Royal Australasian College of Physicians


38 P206

QUESTION 68

A seven-year-old girl is brought into the emergency department with a generalised rash. Her arm is
shown above. This rash is most commonly found in association with:

A. administration of carbamazepine.

B. administration of cefaclor.

C. enterovirus infection.

D. herpes simplex virus (HSV) infection.

E. Mycoplasma pneumoniae infection.

QUESTION 69

A 16-year-old girl is brought to the emergency department by her mother following the ingestion of an
unknown quantity of paracetamol. Four hours post-ingestion her paracetamol serum levels are non-
toxic. She is poorly communicative but states that she does not feel life is worth living anymore and
that she had hoped she would be able to ‘end it all’. Her mother states that the girl has attempted
suicide previously and that she is currently under the care of a private psychiatrist with whom she has
an appointment in one week. The mother feels the girl is overly histrionic and attention seeking. The
girl is attempting to leave the department.

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

A. Detain under the mental health act.

B. Discharge home in care of her mother.

C. Refer back to private psychiatrist as planned.

D. Refer on to community mental health team semi-urgently.

E. Urgent psychiatric review in the emergency department.

Copyright © 2007 by The Royal Australasian College of Physicians


39 P206

QUESTION 70

A six-year-old boy with Down syndrome presents to your practice with persistent diarrhoea for the past
four weeks. Stool microscopy reveals the following findings:

Fat globules: +
Fatty acid crystals: +++
Red blood cells: 0
White blood cells: 0
Faecal pH: 5.5 [> 6.5]
Reducing sugars: 1% [< 0.25%]

What is the most likely diagnosis?

A. Coeliac disease.

B. Cow's milk enteropathy.

C. Crohn disease.

D. Infectious colitis.

E. Lactose intolerance.

QUESTION 71

A 15-year-old girl with chronic eczema presents with facial lesions as shown. The most likely infecting
organism is:

A. Candida albicans.

B. Chlamydia trachomatis.

C. Herpes simplex virus.

D. Herpes zoster virus.

E. Neisseria gonorrhoeae.

Copyright © 2007 by The Royal Australasian College of Physicians


40 P206

QUESTION 72

A six-week-old girl is referred for evaluation of noisy breathing. An extrathoracic narrowing of the
trachea is found on bronchoscopy. The most likely type of noisy breathing in this case is:

A. grunting.

B. rattling.

C. snorting.

D. stridor.

E. wheeze.

QUESTION 73

The electrocardiogram (ECG) shown above was performed on an asymptomatic ten-year-old child
with Ebstein anomaly. The rhythm shown is:

A. atrial fibrillation.

B. atrial flutter.

C. complete heart block.

D. premature atrial contractions.

E. sinus arrhythmia.

Copyright © 2007 by The Royal Australasian College of Physicians


41 P206

QUESTION 74

A six-year-old girl with Down syndrome is seen for annual review. Her parents report she has been
well. She is adjusting to school with some attention difficulties. Her growth charts are shown. She
resists examination. What is the most likely cause for her growth pattern?

A. Coeliac disease.

B. Down syndrome.

C. Growth hormone deficiency.

D. Hypothyroidism.

E. Precocious puberty.

Copyright © 2007 by The Royal Australasian College of Physicians


42 P206

Question 74 (continued)

Copyright © 2007 by The Royal Australasian College of Physicians


43 P206

QUESTION 75

A three-year-old-boy presents with persistent constipation from 18 months of age. He is unresponsive


to laxative therapy. There is no rectal bleeding. His investigations are summarised below.

Haemoglobin 110g/L [110-140]


MCV 68fL [75-90]
WCC 8.3 x 109/L [6.0-17.0]
Total IgA 0.9 g/L [0.26-1.93]
Tissue transglutaminase 45 U/L [0-20]
Thyriod stimulating hormone (TSH) 2.3 mU/L [0.5-4.5]

What is the most likely diagnosis?

A. Coeliac disease

B. Cow's milk allergy

C. Hirschsprung's disease

D. Hypothyroidism

E. Idiopathic constipation

QUESTION 76

A five-year-old boy presents with multiple ulcerated skin lesions (as shown) with raised margins, which
commenced as pustular lesions associated with fever a week prior to presentation. Pyoderma
gangrenosum is confirmed by skin biopsy. Which of the following is most likely to be associated with
this skin lesion?

A. Chronic granulomatous disease.

B. Human immunodeficiency virus (HIV) infection.

C. Inflammatory bowel disease.

D. Pseudomonas septicaemia.

E. Systemic lupus erythematosis.

Copyright © 2007 by The Royal Australasian College of Physicians


44 P206

QUESTION 77

A 13-year-old boy presents to the emergency department after falling out of a hammock onto grass.
He is complaining of a sore left shoulder. On examination there is a tender lump over the left clavicle,
but the overlying skin is not compromised. An X-ray of the area is shown below.

Which of the following is the best next step in management?

A. Broadarm sling.

B. Closed reduction.

C. Figure-of-eight bandage.

D. Open reduction.

E. U-plaster.

QUESTION 78

Which of the following drugs may be taken up to eight hours prior to a histamine bronchial challenge?

A. Montelucast.

B. Salbutamol.

C. Salmeterol.

D. Nedocromil Sodium.

E. Theophylline.

Copyright © 2007 by The Royal Australasian College of Physicians


45 P206

QUESTION 79

A male infant attends at six months of age following relief of neonatal bladder outlet obstruction. The
initial micturating cystourethrogram is shown below.

Which of the following findings is most suggestive of a poor long-term prognosis?

A. Nadir serum creatinine > 0.1 mmol/L.

B. Persistent bladder dilatation.

C. Persistent hydronephrosis.

D. Persistent ureteric reflux.

E. Poor urinary stream.

Copyright © 2007 by The Royal Australasian College of Physicians


46 P206

QUESTION 80

A seven-year-old boy presents with a three month history of staring spells associated with eye
flickering and lip smacking movements. His electroencephalogram (EEG) is shown below.

Which of the following anticonvulsants is most likely to aggravate the underlying seizure disorder in
this patient?

A. Carbamazepine.

B. Clonazepam.

C. Ethosuximide.

D. Sodium valproate.

E. Topiramate.

Copyright © 2007 by The Royal Australasian College of Physicians


47 P206

QUESTION 81

A four-month-old girl with staphylococcal pneumonia acutely deteriorates on the ward with
desaturation and increasing tachypnoea. She is commenced on face-mask continuous positive airway
pressure (CPAP) at 5cm H2O with initial clinical improvement, but again deteriorates with increased
work of breathing, desaturation to 85%, tachycardia (heart rate (HR)=200/minute) and tachypnoea
(respiratory rate (RR)=80/minute). On examination she has decreased air entry and hyperresonance
on the right side of her chest with tracheal deviation to the left. Which of the following should be the
next step in her management?

A. Endotracheal intubation and mechanical ventilation.

B. Increase face mask CPAP to 7cm H2O.

C. Right-sided intercostal catheter.

D. Right-sided needle thoracocentesis.

E. Urgent chest radiograph.

QUESTION 82

A newborn baby of Pacific Islander descent is found to have indeterminate gender. Birth weight was
3200 g and the baby is healthy. The phallus is short and with chordee. A urethra is visible at the base
of the phallic structure. There is a mass in each inguinal canal the size of a testis. The genitalia are
shown above.

The most likely diagnosis is:

A. female with congenital adrenal hyperplasia.

B. male with congenital adrenal hyperplasia.

C. true hermaphrodite.

D. Turner syndrome.

E. undervirilised male.

Copyright © 2007 by The Royal Australasian College of Physicians


48 P206

QUESTION 83

A six-year-old girl presents to the emergency department with a persistent headache and dizziness
following a minor fall one week previously in which there was no loss of consciousness. She has had
several vomits today and feels unwell. On examination, she is afebrile with a respiratory rate of
24/minute, a heart rate of 110/minute and a blood pressure of 96/55 mmHg. She is pale and quiet but
able to respond appropriately to questions and commands. There are no focal neurological signs. Her
non-contrast computed tomography (CT) scan of the head is shown below.

Which of the following is the most likely diagnosis?

A. Extradural haematoma.

B. Intracerebral haemorrhage.

C. Meningioma.

D. Subarachnoid haematoma.

E. Subdural haematoma.

Copyright © 2007 by The Royal Australasian College of Physicians


49 P206

QUESTION 84

A three-week-old term male infant is noticed by his mother to be breathless during feeds. On
examination he is tachypnoeic with a respiratory rate of 90 breaths per minute, marked subcostal
recession, and poor breath sounds on the left side. The chest X-ray is shown below.

The most likely diagnosis is:

A. congenital cystic adenomatoid malformation.

B. congenital lobar emphysema.

C. dextrocardia.

D. diaphragmatic hernia.

E. pneumothorax.

Copyright © 2007 by The Royal Australasian College of Physicians


50 P206

QUESTION 85

A newborn baby is noticed to have genital ambiguity. Gonads are palpable in the labioscrotal region.
Investigation reveals XY Karyotype. Following HCG (human chorionic gonadotrophin) stimulation test
at two weeks the maximum testosterone level is 6.6 nmol/L [<1.7].

The most likely diagnosis is:

A. androgen insensitivity syndrome.

B. congenital adrenal hyperplasia.

C. gonadal dygenesis.

D. Klinefelter syndrome.

E. testosterone biosynthetic defect.

QUESTION 86

A test has a sensitivity of 95% and a specificity of 90%. It is used to screen the general population for
a condition that has a prevalence of 1 in 100,000.

What will the positive predictive value be nearest to?

A. 0.01%.

B. 0.05%.

C. 0.1%.

D. 0.5%.

E. 1%.

QUESTION 87

A four-year-old girl presents for investigation of developmental regression. Early development had
been normal, but starting from nine months of age she had lost social and motor skills, and by the age
of two years had almost no purposeful hand movements. By the age of four years, she exhibited
stereotyped behaviours and frequent episodes of hyperventilation. She has a history of two
generalised seizures.

The most likely diagnosis is:

A. autism.

B. Leigh disease.

C. metachromatic leukodystrophy.

D. neuronal ceroid lipofuscinosis.

E. Rett syndrome.

Copyright © 2007 by The Royal Australasian College of Physicians


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QUESTION 88

An 18-month-old baby of consanguineous parents of Muslim faith is found to be delayed in his motor
milestones. In particular he is not walking. He is breast fed, and tolerates feeds well. He is the third
child in the family and his cognitive development is similar to his two older siblings.

The following investigations are done:

25-Vitamin D 16 nmol/L [30-150]


Calcium 2.23 mmol/L [2.15-2.60]
Phosphate (PO4) 1.82 mmol/L [1.10-1.80]
Parathyroid hormone 14 pmol/L [0.8-8.0]
Maternal vitamin D 21 nmol/L [35-150]

The most likely cause of these findings is:

A. Familial hyperphophataemic rickets.

B. Nutritional vitamin D deficiency.

C. Primary hyperparathyroidism.

D. Pseudohypoparathyroidism.

E. Vitamin D resistant rickets.

QUESTION 89

A three-year-old girl is seen with a history of a 24-hour viral illness. She is brought to the hospital
following a 15-minute generalised seizure. Blood glucose is 2.6 mmol/L [4.5-6.2]. Further
investigations are as follows:

Insulin 1 mU/L [<12]


Beta hydroxy butyrate 2.4 mmol/L [<0.3]
Ammonium 35 umol/L [<50]

What is the most likely diagnosis in this child?

A. Glucokinase enzyme mutation.

B. Glutamate dehydrogenase enzyme mutation.

C. Ketotic hypoglycaemia.

D. Malabsorption.

E. Maple syrup urine disease.

Copyright © 2007 by The Royal Australasian College of Physicians


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QUESTION 90

An 11-year-old girl has undergone a T-cell depleted unrelated bone marrow transplant for relapsed
acute lymphoblastic leukaemia. Recovery is complicated by slow neutrophil engraftment, grade 3
acute graft-versus-host disease requiring high dose methylprednisolone, and persistent fevers. The
computerised tomography (CT) scan of her chest, performed at day +50 post-transplantation, is shown
above.

Which one of the following pathogens is most likely to be responsible for the CT scan findings?

A. Aspergillus fumigatus.

B. Candida albicans.

C. Klebsiella pneumoniae.

D. Scedosporium prolificans.

E. Staphylococcus aureus.

Copyright © 2007 by The Royal Australasian College of Physicians


53 P206

QUESTION 91

Following an uneventful labour and delivery, a term female infant is born in good condition. At six
hours of age, she is noted to be cyanosed. She is vigorous with a pulse rate of 140/minute, and no
signs of respiratory distress or heart murmur. Her pulses are normal volume. A pulse oximeter placed
on her right hand reveals an oxygen saturation of 75%. The most likely diagnosis is:

A. hypoplastic left heart.

B. persistent pulmonary hypertension of the newborn.

C. tetralogy of Fallot.

D. total anomalous pulmonary venous drainage.

E. transposition of the great vessels.

QUESTION 92

The mother of a ten-year-old with type 1 (insulin dependent) diabetes mellitus asks for your advice
about a breakfast cereal, recommended as "good for diabetics" by a health food shop.

The cereal Nutrition Information Panel is shown.

Based on this information you advise that the cereal is not a good choice for her child because it is:

A. high in sugar, although low in fat and high in fibre.

B. high in sugar and high in fat, although high in fibre.

C. high in sugar, high in fat and low in fibre.

D. low in sugar and low in fat, but also low in fibre.

E. low in sugar, but high in fat and low in fibre.

Copyright © 2007 by The Royal Australasian College of Physicians


54 P206

QUESTION 93

A 15-year-old boy is seen with concerns about his lack of pubertal development. On examination his
height is on the third percentile, pubic hair Tanner stage 1, genital development Tanner stage 1 and
testes 6ml bilaterally. His bone age is 12-years- and-six-months.

The most likely diagnosis is:

A. constitutional growth delay.

B. growth hormone deficiency.

C. hypergonadotrophic hypogonadism.

D. hypogonadotrophic hypogonadism.

E. Kallman syndrome.

QUESTION 94

The principle of informed consent relates to which ethical standard?

A. Autonomy.

B. Beneficence.

C. Justice.

D. Non-maleficence.

E. Privacy.

Copyright © 2007 by The Royal Australasian College of Physicians


55 P206

QUESTION 95

A 4.9kg term infant is delivered by emergency caesarian section for foetal distress. His mother had no
antenatal care. He requires four minutes of bag and mask ventilation but by 10 minutes of age has an
Apgar score of 9. He tolerates a bottle feed of 45ml of milk formula at 60 minutes of age. Laboratory
blood sugar is 0.9 mmol/L at four-hours of age and he is admitted to the Neonatal Intensive Care Unit
(NICU). On examination he is macrosomic, hirsute and has a large tongue but no other dysmorphic
features.

Laboratory investigations include:

Blood glucose 1.2 mmol/L [> 2.6]


Plasma cortisol 350 nmol/L [250-800]
Plasma growth hormone 43.4 µg/L [5-53]
Plasma insulin 80 pmol/L [9-80]
beta-hydroxybutyrate 20 µmol/L [20-270]
Free fatty acids 0.0 mEq/L [0.0-0.6]
Plasma ammonia 91 µmol/L [<100]
Blood pyruvate 151 µmol/L [30-165]
Urinary ketones negative

Which of the following is the most likely cause of the hypoglycaemia?

A. Adrenal insufficiency.

B. Fatty acid oxidation defect.

C. Hyperinsulinism.

D. Hypopituitarism.

E. Simple substrate deficiency.

Copyright © 2007 by The Royal Australasian College of Physicians


56 P206

QUESTION 96

A six-month-old boy presents to the emergency department with a five day history of coryza, low grade
fever and poor oral intake. He has developed an extensive migratory rash overnight and is generally
miserable. On examination he has a widespread rash as shown below, with no evidence of mucous
membrane involvement. He has been previously well and is not on any regular medications.

Which of the following is the most likely diagnosis?

A. Erythema marginatum.

B. Erythema multiforme.

C. Stevens-Johnson syndrome.

D. Urticaria.

E. Viral exanthem.

QUESTION 97

A three-year-old boy presents after developing severe swelling of the left foot and leg to mid thigh
immediately after being stung by a bee on the left foot. There is a past history of large local reactions
to mosquito bites. He has mild eczema and wheezes in association with viral upper respiratory tract
infections. His risk of a severe systemic reaction to a subsequent bee sting is closest to:

A. 10%.

B. 30%.

C. 50%.

D. 70%.

E. 90%.

Copyright © 2007 by The Royal Australasian College of Physicians


57 P206

QUESTION 98

A seven-day-old term infant is noted to have a bilateral purulent eye discharge which is yellow in
colour. There is associated cellulitis of both eyelids. The most likely cause of this infant's conjunctivitis
is:

A. Chlamydia trachomatis.

B. Neisseria meningitidis.

C. Herpes simplex virus.

D. Pseudomonas aeruginosa.

E. Staphylococcus aureus.

QUESTION 99

A three-year-old boy presents to the emergency department with a fever and coryza. On examination
he has a temperature of 37.8°C, heart rate 110/minute, respiratory rate 30/minute, blood pressure
88/60 mmHg. He is miserable with a runny nose and palpable small cervical nodes. He has bilaterally
red tympanic membranes which are not bulging and his pharynx is slightly inflamed with no purulent
exudate. His chest and abdominal examination are unremarkable and he has no evidence of oedema.
His urinalysis demonstrates 3+ haematuria, with no proteinuria. A spun urine demonstrates the
following:

Result
Glucose Negative
Bilirubin Negative
Ketones Negative
Specific Gravity 1.010
Blood 2+
pH 7.0
Protein Negative
Urobilinogen Negative
Nitrites Negative
Leukocytes Negative
Red Blood Cell Number/High Power Field 2-3
Dysmorphic Red Blood Cell 20%
Casts Number/Low Power Field 0
Casts Number/Slide 0
White Blood Cell Number/High Power Field 0
Other Cells None Seen
Bacteria None Seen

Which of the following is the most appropriate next investigation?

A. Coagulation studies.

B. Renal ultrasound.

C. Repeat urinalysis in 3-6 months.

D. Serum creatinine.

E. Urinary calcium / creatinine ratio.

Copyright © 2007 by The Royal Australasian College of Physicians


58 P206

QUESTION 100

A term male infant is admitted to hospital on day seven of life for repair of an inguinal hernia. He is the
first child to non-consanguineous parents. His mother is quite anxious, and reports that her son has
not been feeding particularly well over the past two days. He is afebrile and his clinical examination
reveals a reducible right sided inguinal hernia. Otherwise his examination is normal. The pre-operative
chest X-ray arranged by the anaesthetists is shown below.

The chest X-ray is most consistent with:

A. cardiomegaly.

B. congenital neuroblastoma.

C. extra-pulmonary sequestration.

D. normal thymic shadow.

E. right upper lobe consolidation.

Copyright © 2007 by The Royal Australasian College of Physicians


59 P206

2006 FRACP Written Examination

Paediatrics & Child Health

Paper 2 – Clinical Applications

Answers
1. B 34. C 67. A
2. D 35. E 68. D
3. B 36. D 69. A
4. D 37. E 70. A
5. A 38. C 71. C
6. A 39. D 72. D
7. D 40. E 73. B
8. C 41. D 74. D
9. B 42. E 75. A
10. C 43. B 76. C
11. A 44. E 77. A
12. C 45. C 78. B
13. D 46. C 79. A
14. A 47. B 80. A
15. D 48. D 81. D
16. B 49. C 82. E
17. D 50. E 83. A
18. C 51. D 84. B
19. E 52. B 85. A
20. A 53. E 86. A
21. B 54. B 87. E
22. D 55. C 88. B
23. B 56. A 89. C
24. B 57. A 90. A
25. E 58. C 91. E
26. D 59. E 92. B
27. E 60. B 93. A
28. D 61. B 94. A
29. B 62. C 95. C
30. B 63. C 96. D
31. A 64. C 97. A
32. E 65. A 98. A
33. A 66. B 99. C
100. D

Copyright © 2007 by The Royal Australasian College of Physicians

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