Model Paper One

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Model paper one

Question 1.

A rhythm strip from an electrocardiogram (ECG) is shown above. Which of the


following is the most likely cause for the abnormality displayed?
A. Hyperkalaemia.
B. Hyperthermia.
C. Hyperthyroidism.
D. Hypocalcaemia.
E. Uraemia.

2. Steroids used in the treatment of childhood acute lymphoblastic leukaemia are


least frequently associated with which one of the following side effects?
A. Avascular necrosis of bone.
B. Behavioural disturbances.
C. Bone demineralisation.
D. Renal tubular defects.
E. Weight gain.

3. The most important known factor which influences the rate of development of
microvascular complications in patients with diabetes is:
A. duration of diabetes.
B. hyperglycaemia.
C. hyperlipidaemia.
D. hypertension.
E. smoking.

4. Which of the following anticonvulsants is most likely to increase the half-life of


topiramate?
A. Carbamazepine.
B. Phenobarbitone.
C. Phenytoin.
D. Primidone.
E. Sodium valproate.

5. Which of the following antibiotics would be expected to be least active in


treating a patient with infection caused by Pseudomonas aeruginosa?
A. Cefotaxime.
B. Gentamicin.
C. Imipenem.
D. Piperacillin.
E. Ticarcillin.

6. A 24-day-old boy presents with a one-week history of projectile vomiting and


weight loss. Which of the following findings would be most likely on arterial blood
gas measurement?
A. Metabolic acidosis.
B. Metabolic alkalosis.
C. Mixed metabolic acidosis and respiratory alkalosis.
D. Mixed metabolic alkalosis and respiratory acidosis.
E. Normal blood gas.

7. In a child with a massive abdominal Burkitt lymphoma, the most useful


treatment to prevent tumour lysis syndrome is:
A. allopurinol.
B. frusemide.
C. mannitol.
D. prednisolone.
E. urate oxidase.

8. Idiopathic ascending aortic dilatation would be most likely to accompany


which of the following syndromes/associations?
A. Down syndrome.
B. Noonan syndrome.
C. Turner syndrome.
D. VACTERL association.
E. Velocardiofacial syndrome.

9. A breastfed two-day-old girl presents with profuse diarrhoea and develops


severe dehydration. The diarrhoea ceased during resuscitation with intravenous
(IV) fluids when feeds were paused. However, when breastfeeds were gradually
introduced in the recovery period, the diarrhoea returned and IV fluids were
recommenced. A trial of oral rehydration solution also resulted in severe
diarrhoea. Analysis of the faecal fluid revealed the following electrolyte profile:
sodium 20 mmol/L
potassium 15 mmol/L
chloride 25 mmol/L
Which one of the following is the most likely diagnosis?
A. Congenital chloride diarrhoea.
B. Glucose-galactose malabsorption.
C. Primary lactose intolerance.
D. Secondary lactose intolerance.
E. Sucrase-isomaltase deficiency.

10. The daily energy requirement of a parenterally fed three-week-old neonate is


estimated to be 100 kcal (420 kJ) per kilogram body weight. Which of the
following intravenous glucose and lipid solutions will provide adequate daily
energy for an infant of 4 kg body weight?

A. 400 mL of glucose 10% plus 20 mL of intravenous lipid 20%.


B. 400 mL of glucose 15% plus 30 mL of intravenous lipid 20%.
C. 400 mL of glucose 15% plus 40 mL of intravenous lipid 20%.
D. 400 mL of glucose 15% plus 50 mL of intravenous lipid 20%.
E. 400 mL of glucose 20% plus 60 mL of intravenous lipid 20%.

11. Which of the following antipsychotic medications is most likely to cause the
greatest weight increase when prescribed to adolescents?
A. Haloperidol.
B. Olanzapine.
C. Pimozide.
D. Quetiapine.
E. Risperidone.

12. Low serum vitamin B12 levels are least likely to be seen in which one of the
following?
A. Crohn disease.
B. Pernicious anaemia.
C. Severe folate deficiency.
D. Short bowel syndrome.
E. Transcobalamin II deficiency.

13. The word complex in complex partial seizures refers to:


A. a seizure lasting >10 minutes.
B. automatisms occurring during the seizure.
C. focal motor seizures.
D. impaired consciousness during the seizure.
E. multiple seizures in a 24-hour period.

14. The most effective way of preventing mother-to-child transmission of human


immunodeficiency virus (HIV) is:
A. antiretroviral therapy to the mother during delivery.
B. antiretroviral therapy to the mother during pregnancy.
C. antiretroviral therapy to the newborn baby.
D. bottle-feeding.
E. Caesarean section.

15. Which of the following most accurately describes the known effects of
supplementation of artificial milk formulae with long chain polyunsaturated fatty
acids (LCPUFA) in term infants?
A. A beneficial effect on bone maturation.
B. A beneficial effect on growth.
C. A beneficial effect on neurodevelopmental outcome.
D. A beneficial effect on visual maturation.
E. No additional benefit demonstrated.

16. If two or more first-degree relatives have atopic disorders, the risk of a
subsequent infant developing an atopic disorder is closest to:
A. <10%.
B. 20%.
C. 33%.
D. 75%.
E. 90%.

17. Activated protein C resistance is most likely to be associated with which one
of the following states?
A. Antithrombin III deficiency.
B. Factor V Leiden mutation.
C. Factor VIII deficiency.
D. Lupus anticoagulant.
E. Prothrombin gene mutation.
18. A six-year-old patient with coeliac disease undergoes dual sugar intestinal
permeability testing. Two sugars, rhamnose and lactulose, are administered
orally and the subsequent urinary excretion is measured over five hours.
Rhamnose is actively absorbed by the small intestinal villus, whereas lactulose is
absorbed via passive paracellular transport. Which of the following findings in
urine indicate poor adherence to a gluten-free diet?
Rhamnose Lactulose

A. Decreased Decreased

B. Decreased Normal

C. Increased Decreased

D. Increased Normal

E. Normal Increased

19. An ambulance attends a 13-year-old girl who collapses at school and is


unconscious for a brief period. Witnesses to the event said that she had some
brief jerking of her limbs when on the ground. By the time she is brought to the
emergency room she is back to normal. On further questioning, there have been
three previous events.
An association with which of the following is most likely to raise concerns that
the diagnosis is not simple syncope?
A. Exercise.
B. Mild trauma.
C. Prolonged standing.
D. Venipuncture.
E. Vomiting.

20. Among children and adolescents with significant behavioural disturbances, a


poor prognosis is most likely with which of the following?
A. Alcoholic or sociopathic father.
B. Greater severity of problem behaviour.
C. Lower age of presentation.
D. Maternal psychosocial problems.
E. Parental divorce.

21. A 12-year-old girl completed treatment for a Ewing sarcoma four years ago.
The chemotherapy agent most likely to increase her risk of second malignancy
is:
A. actinomycin-D.
B. cyclophosphamide.
C. doxorubicin.
D. methotrexate.
E. vincristine.

22. Which one of the following interventions has most consistently been shown
to decrease the duration of hospital stay with acute asthma?
A. Inhaled ipratropium bromide.
B. Inhaled salbutamol.
C. Intravenous salbutamol.
D. Oral prednisolone.
E. Oral theophylline.

23. A 15-year-old boy has been on an anticonvulsant for three years. The dosage
has been stable. He recently noticed difficulties in seeing at night. A formal
ophthalmological assessment demonstrated visual field constriction.
Which one of the following anticonvulsants is most likely to be the cause of his
symptoms?
A. Carbamazepine.
B. Gabapentin.
C. Lamotrigine.
D. Sodium valproate.
E. Vigabatrin.

24. A term neonate presents with jitteriness at three hours of age. The following
recordings are made:
weight 3450 g (50th percentile)
length 50 cm (50th percentile)
head circumference 36 cm (50th percentile)
penile length 1.8 cm (<10th percentile)
plasma glucose 0.7 mmol/L
The remainder of the examination is normal.
The most likely cause is:
A. congenital adrenal hyperplasia.
B. 5 alpha-reductase deficiency.
C. hyperinsulinism.
D. hypopituitarism.
E. 45XO/46XY mosaicism.

25. The following results are obtained from a 14-year-old boy, prior to elective
hernia surgery, who gives a history of excessive bleeding after dental extraction.
Bleeding time 6 minutes [2-9]
Prothrombin time 12 seconds [12-14]
Prothrombin time-international normalised ratio (PT-INR) 1.0 [0.9-1.2]
Activated partial thromboplastin time (APTT) 56 seconds [26-35]
Fibrinogen 2.4 g/L [1.8-4.0]
Mixing the patient's plasma with an equal volume of normal plasma
normalises the APTT.
Which one of the following is the most likely diagnosis?
A. Antiphospholipid antibody syndrome.
B. Factor VII deficiency.
C. Haemophilia.
D. Recent aspirin ingestion.
E. Von Willebrand disease.

26. Second generation H1 receptor antagonists effectively relieve most of the


symptoms of allergic rhinoconjunctivitis.
Which one of the following symptoms is least effectively relieved?
A. Nasal congestion.
B. Nasal itch.
C. Ocular tearing.
D. Rhinorrhoea.
E. Sneezing.

27. A six-month-old girl develops a chylothorax post-cardiac surgery. After two


days her chest drain continues to drain chylous fluid.
The most appropriate feed for the management of chylothorax in this infant is:
A. breast milk.
B. Isomil (soy milk formula).
C. Neocate (single amino acid infant formula).
D. Portagen (casein hydrolysate formula).
E. S26 (whole protein cows milk formula

28. Which one of the following vaccines is contraindicated in a child with


anaphylactic egg allergy?
A. Bacille Calmette-Gurin (BCG).
B. Influenza.
C. Measles-mumps-rubella (MMR).
D. Oral polio.
E. Whole cell pertussis.

29. An 18-month-old boy has recurrent blue breath-holding episodes, followed on


one occasion by a 15-second generalised seizure.
The investigation most likely to be useful in directing therapy is:
A. electrocardiogram (ECG).
B. electroencephalogram (EEG).
C. iron studies.
D. plasma calcium.
E. plasma glucose.

30. A three-year-old boy presents with a six-month history of polyarticular


arthritis. He had been well until age 12 months when he required hospitalisation
for left lower lobe pneumonia. Recurrent otitis media, intermittent fevers and
persistent diarrhoea developed in the second year of life. There was no history of
oral thrush or other fungal infections and no history of opportunistic infections.
Developmental milestones and physical growth were normal and immunisations
were appropriate for age.
Physical examination shows a febrile boy who refuses to weight bear. He has
purulent nasal discharge. The lung fields are clear. Both knees are swollen,
tender and warm with effusions and decreased range of movement. Both ankles
are tender with synovial swelling and there is a reduced range of movement.
Examination of the heart is normal. Skin examination is normal.
Which one of the following is the most likely diagnosis?
A. Chronic meningococcaemia.
B. Juvenile chronic arthritis.
C. Late onset hypogammaglobulinaemia (common variable immunodeficiency).
D. Rheumatic fever.
E. Yersinia arthritis.

31. Which feature distinguishes hyper-IgE syndrome from severe atopic


dermatitis?
+
A. Decreased number of peripheral blood CD8 lymphocytes.
B. Distribution of the eczematous rash.
C. Extremely high serum IgE levels.
D. Negative delayed skin tests to Candida.
E. Staphylococcal abscesses in the axillary lymph nodes.

32. A four-year-old child has a pneumonia with an effusion that is unresponsive to


antibiotic therapy given intravenously in appropriate doses (ceftriaxone,
tobramycin and flucloxacillin) and formal surgical drainage of the
effusion/empyema. Five days after the surgery the child still has temperatures
greater than 39C and looks unwell. Bacteroides fragilis is grown from the
effusion.
A change to which one of the following antibiotics is likely to improve the child?
A. Ceftazidime.
B. Clindamycin.
C. Erythromycin.
D. Penicillin G.
E. Vancomycin.

33. You are asked for a second opinion regarding a 10-year-old boy who has a
long history of hyperactive, impulsive, attention-seeking and destructive
behaviour, both at home and at school. A paediatrician and a child psychiatrist
have previously diagnosed him as having attention deficit hyperactivity disorder
(ADHD). He responded poorly to dexamphetamine but concentration and
attention have improved on methylphenidate. He is currently on 30 mg/day. His
weight is 35 kg.
Further assessment demonstrated average intelligence but with a significant
language-based learning disorder. He is at the 3rd percentile for receptive
language and the 14th percentile for expressive language. He is making progress
at school with the provision of remedial help and an integration aide.
He also receives on-going counselling and cognitive behavioural management
from his psychiatrist.
His parents are divorced and are unable to manage him consistently. His father is
strict, distant and loses his temper with his son. His mother is caring, finds it
hard to set limits and is overwhelmed by his demands. The boy dominates and
intrudes on all aspects of family life. His father denigrates his ex-wife in front of
the boy.

Which one of the following is likely to be the most helpful?


A. Alter medication regime.
B. Family therapy.
C. Foster care.
D. Individual parental guidance.
E. Transfer to a special school.

34. A 14-month-old infant has been dependent on parenteral nutrition since birth
due to surgical resection secondary to volvulus of the small bowel. He currently
receives 30% of his caloric requirement by the parenteral route and the
remainder as Pregestimil via a nasogastric tube. On examination he is icteric.
He has 3 cm of splenomegaly, the liver is not palpable and there is no ascites.
His blood tests reveal:
bilirubin (total) 120 mol/L [<10]
bilirubin conjugated 96 mol/L [<10]
alanine aminotransferase (ALT) 203 U/L [10-50]
gamma glutamyltransferase (GGT) 593 U/L [5-24]
albumin 23 g/L [34-52]
Abdominal ultrasound shows a small liver with normal intra and extra hepatic
ducts and an enlarged spleen. A Doppler study of his portal vessels shows blood
flowing from the liver to the spleen. The most effective intervention to arrest the
progression of his liver disease would be to:
A. administer chenodeoxycholic acid.
B. administer phenobarbitone.
C. administer rifampicin.
D. cease parenteral nutrition.
E. perform a portacaval shunt.

35. A four-year-old boy presents with the sole symptom of a generally dry cough
of four months duration, which has been affecting his sleep. He is well grown and
there are no localising or generalised signs. He has not responded to nebulised
salbutamol prescribed by his general practitioner. His blood count and chest X-
ray are normal.
Which one of the following is the most appropriate management?
A. Bedroom humidification.
B. Erythromycin.
C. Inhaled corticosteroids.
D. No therapy.
E. Oral steroids.

36. A four-year-old child presents to the emergency department with a two-day


history of multiple bruises and a bleeding nose, two weeks after an upper
respiratory tract infection. His past medical history is unremarkable. On
examination, in addition to the features described in the history, there is a
widespread petechial rash noted mainly over the trunk but there are no other
abnormal features. Full blood count shows the following results:
haemoglobin 117 g/L [110-150]
mean corpuscular volume 79 fL [75-90]
red cell morphology normal
9
platelet count <10 x 10 /L [150-400]
9
white cell count 9.8 x 10 /L [5.0-14.5]
differential:
9
lymphocytes 5.8 x 10 /L [1.5-10.0]
9
neutrophils 3.8 x 10 /L [1.0-8.0]
9
monocytes 0.2 x 10 /L [0.2-1.2]
Which one of the following treatments will result in the most rapid rise in the
platelet count?
A. Anti-D immunoglobulin.
B. Danazol.
C. Dexamethasone.
D. Intravenous immunoglobulin.
E. Prednisolone.

37. Which one of the following factors is most likely to be associated with the
occurrence of late-onset haemorrhagic disease (vitamin K deficiency bleeding)?
A. Breastfeeding.
B. Formula feeding.
C. Maternal anticonvulsants.
D. Post-natal antibiotic administration.
E. Prematurity.
38. A previously well two and a half-year-old girl, whose parents are divorced,
returns from weekend access with her father. On return home the child is a little
more demanding than usual but is otherwise behaviourally normal. At bed-time
the mother notices that her daughter has a significantly reddened vulva.
Which one of the following is the most likely diagnosis?

A. Candidiasis.
B. Child sexual abuse.
C. Lichen sclerosis.
D. Non-specific vulvovaginitis.
E. Straddle injury.

39. A 15-year-old girl presents with a three-year history of school avoidance,


attending approximately one week per month. She held a scholarship in
secondary school but this was withdrawn due to poor attendance.
At home, she spends her day watching television, drinking alcohol or smoking
marijuana. She has a few friends but is reluctant to leave the house. She is
preoccupied with her body and feels herself to be a freak because she perceives
asymmetry in her facial appearance. She panics in social situations, believing
that other people see this appearance. She is volatile and flies into a rage if she
is thwarted. She hates herself, has grazed her wrists and there are scars from
cigarette burns on her arms. She denies suicidal ideation.
At night, she finds it difficult to fall asleep, worries about being attacked and
often checks the door and window locks. She has frequent nightmares and on
waking, she thinks she hears strangers in her room.
Her parents divorced eight years ago and do not get on well. She lives with her
mother and brother, the latter having similar but less severe difficulties. He is
now at university. Her mother is anxious and ineffectual and it is clear that the
children control the family.
Which one of the following diagnoses best explains this picture?
A. Affective disorder (depression).
B. Borderline personality disorder.
C. Obsessive-compulsive disorder.
D. Panic disorder.
E. Schizophrenia.

40. Which one of the following would not be consistent with a diagnosis of night
terrors?
A. Associated somnambulism.
B. Associated sweating and pupillary dilatation.
C. Inability to recall the event.
D. Multiple episodes per night.
E. Occurrence at age seven years.

41. A 13-year-old boy is brought to the emergency room following an intentional


ingestion of battery acid 15 minutes previously. He complains of abdominal pain.
On examination he is pale with a pulse rate of 100/minute, respiratory rate of
30/minute and a systolic blood pressure of 120 mmHg. He has upper abdominal
tenderness with no obvious rebound tenderness. Bowel sounds are present and
normal in character.
Which one of the following is the most appropriate immediate management?
A. Administer antacid.
B. Administer corticosteroids.
C. Administer ipecac.
D. Insert a nasogastric tube and suction gastric contents.

E. Perform a gastroscopy.

42. A nine-month-old boy, who has been fully immunised, presents with a
pneumonia and has the following results:
IgG 1.1 g/L [2.1-12.2]
IgA <0.10 g/L [0.17-1.20]
IgM 0.15 g/L [0.32-1.40]
IgE <5 kU/L [0-35]
Lymphocyte markers:
CD3 (T cells) 93% [53-71]
CD4 (T helper) 68% [28-52]
CD8 (T suppressor) 24% [13-31]
CD19 (B cells) 0% [19-38]
natural killer (NK) cells 4% [3-12]
His pneumonia is most likely to be due to which one of the following organisms?
A. Escherichia coli.
B. Haemophilus influenzae type b.
C. Mycoplasma pneumoniae.
D. Pneumocystis carinii.
E. Staphylococcus aureus

43. An eight-month-old breast-fed baby girl is brought to the emergency


department with a 10-day history of vomiting, irritability and weight loss. The
baby developed normally until six months of age but since then her development
has regressed and she is no longer able to sit unsupported.
She is afebrile, pale and listless. Her weight is 6.5 kg (3-10th percentile), length
68 cm (50th percentile) and head circumference is 43 cm (10-50th percentile).
She is generally hypotonic and has brisk reflexes with positive Babinski
responses. Abdominal examination is normal. Investigations show:
haemoglobin 98 g/L [95-140]
mean corpuscular volume 106.5 fL [70.0-85.0]
mean corpuscular haemoglobin 34.9 pg [24.0-36.0]
white cell count 5.8 x 109/L [5.0-17.0]
neutrophils 1.1 x 109/L [1.0-8.0]
occasional hypersegmented neutrophils seen on blood film
platelet count 445 x 109/L [150-600]
sodium 145 mmol/L [135-145]
chloride 99 mmol/L [95-110]
potassium 4.5 mmol/L [3.5-5.5]
bicarbonate 26 mmol/L [22-26]
urea 6.9 mmol/L [1.3-6.6]
creatinine 0.045 mmol/L [0.020-0.050]
glucose 4.3 mmol/L [2.5-5.5]
Urine metabolic screen gross increase in methylmalonic acid and homocystine
Which one of the following is the most likely diagnosis?
A. Cobalamin C deficiency.
B. Glutaric aciduria type II.
C. Homocystinuria.
D. Methylmalonic acidaemia.
E. Vitamin B12 deficiency.

44. Which anti-arrhythmic drug is inappropriate in the treatment of the


arrhythmia with which it is paired?
A. Atrial flutter: sotalol.
B. Atrial tachycardia in Wolff-Parkinson-White syndrome: flecainide.
C. Prolonged QT syndromes: propranolol.
D. Sinus node dysfunction: digoxin.
E. Ventricular tachycardia in cardiomyopathy: amiodarone.

45. A 30-year-old mother and her five-year-old son are both HIV (human
immunodeficiency virus)-positive. She is well and takes all her own medication
reliably. She is pregnant and would like to know what are the chances of her baby
developing HIV infection. She understands that both baby and she will be treated
with the best available current treatments.
The risk of the baby acquiring HIV is closest to:
A. 90%.
B. 70%.
C. 50%.
D. 30%.
E. 10%.

46. Stevens-Johnson syndrome is most likely to occur with which one of the
following anticonvulsant drugs?
A. Carbamazepine.
B. Lamotrigine.
C. Phenytoin.
D. Sodium valproate.
E. Vigabatrin.

47. A 10-year-old girl presents with a recurrent persistent distressing cough of


four months duration. The cough does not occur during sleep, but starts on
awakening and fluctuates in intensity and frequency throughout the day. Her
mother describes the cough as very loud. The cough has been present daily and
it started with an upper respiratory tract infection. There was a similar episode
12 months ago at the same time of the year. The cough was not associated with
wheeze, dyspnoea or any sputum production. Her chest X-ray is normal.
Which one of the following is the most likely diagnosis?
A. Cough variant asthma.
B. Episodic asthma.
C. Pertussis.
D. Post-viral chronic bronchitis.
E. Psychogenic cough.

48. You have been asked to review the biochemical profile of a 13-year-old girl
who is undergoing nasogastric feeding for severe anorexia nervosa, which was
initiated three days earlier. You calculate that she is receiving 100 calories per
hour. She had normal biochemistry on admission.
Her current biochemical profile is:
sodium 135 mmol/L [134-142]
potassium 2.7 mmol/L [3.5-4.5]
chloride 98 mmol/L [96-110]
urea 1.0 mmol/L [2.1-6.5]
creatinine 0.02 mmol/L [0.03-0.08]
glucose 2.4 mmol/L [3.5-5.4]
calcium 1.99 mmol/L [2.10-2.60]
phosphate 0.8 mmol/L [1.1-1.8]
albumin 30 g/L [35-50]
This picture is most likely to be due to which one of the following?
A. Addisonian crisis.
B. Diuretic abuse.
C. Laxative abuse.
D. Secondary renal tubular acidosis.
E. The enteral nutrition.

49. Which one of the following cardiac lesions would be an unexpected finding in
a baby with a 22q11 deletion?
A. Infradiaphragmatic totally anomalous pulmonary venous return.
B. Interrupted aortic arch.
C. Pulmonary atresia, ventricular septal defect and major aorto-pulmonary
collaterals.
D. Tetralogy of Fallot.
E. Truncus arteriosus.

50. Which one of the following measures is most effective in preventing neonatal
early onset group B streptococcal infection?
A. Antibiotics (single intramuscular dose) given after delivery to babies of
colonised mothers.
B. Antibiotics given during pregnancy to colonised mothers at 28 weeks
gestation.
C. Antibiotics given during pregnancy to colonised mothers with group B
streptococcal antigenuria.
D. Maternal intrapartum antibiotics to colonised mothers.
E. Vaginal antiseptic douches before and during delivery to intrapartum colonised
mothers.

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