1999p1 fracp written examination paediatrics & child
TRANSCRIPT
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1999 FRACP Written Examination
Paediatrics & Child Health
Paper 1 Medical Sciences
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 visualmaterial.
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 Executive Officer, Examinations Section, Department of Training and Assessment via e-mail:
Please note that with changes in medical knowledge, some of the information may no longer be current.
Copyright 2003 by The Royal Australasian College of Physicians
All Written Examination papers are copyright. They may not be reproduced in whole or part without writtenpermission from The Royal Australasian College of Physicians, 145 Macquarie Street, Sydney, Australia.
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Question 1
A three-year-old girl has a large secundum atrial septal defect.
The magnitude of the left-to-right atrial shunt will be most influenced by the:
A. left atrial pressure.
B. left ventricular compliance.
C. pulmonary vascular resistance.
D. right ventricular compliance.
E. systemic vascular resistance.
Question 2
A 12-year-old boy falls off his bike and breaks his left humerus. He complains of numbness on the back of his
hand. On examination he has a left wrist drop, and sensory abnormalities over the posterior aspect of his handand in the anatomical snuff box.
The nerve involved is the:
A. dorsal cutaneous.
B. median.
C. musculocutaneous.
D. radial.
E. ulnar.
Question 3
Drug X is given intravenously using two different regimens, to the same patient on two different occasions. Both
times there is no measurable amount of the drug in plasma prior to its administration. On both occasions the
volume of distribution of X is 0.85 L/kg and the plasma half-life is six hours.
Regimen A: Continuous intravenous infusion of 10 mg/kg/hr, with no loading dose.
Regimen B: A loading dose of 50 mg/kg given rapidly intravenously, then a continuous
intravenous infusion of 10 mg/kg/hr.
Using regimen A, the plasma concentration of X after 48 hours was 100 mg/L.
One compartment, first order pharmacokinetics can be assumed.
The plasma concentration of X, after 48 hours, using regimen B is closest to:
A. 50 mg/L.
B. 75 mg/L.
C. 100 mg/L.
D. 125 mg/L.
E. 150 mg/L.
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Question 4
Which one of the following diseases is not considered to be mediated by antigens acting as "super-antigens"?
A. Kawasaki disease.
B. Rheumatic fever.
C. Scarlet fever.
D. Staphylococcal toxic shock syndrome.
E. Streptococcal toxic shock syndrome.
Question 5
Collaboration involving physical contact between B and T cells is essential for the activation of B cells and
immunoglobulin class switching. Contact occurs between CD40 on the surface of the B cell and the CD40 ligand
on activated T cells.
In which one of the following is CD40 ligand binding most likely to be defective?
A. Selective IgA deficiency.
B. Severe combined immunodeficiency.
C. Wiskott-Aldrich syndrome.
D. X-linked agammaglobulinaemia.
E. X-linked hyper IgM syndrome.
Question 6
A five-year-old boy was successfully treated with intensive chemotherapy for acute lymphoblastic leukaemia. Four
years later, however, he is diagnosed with a second malignancy of non-Hodgkins lymphoma.
Which one of the following chemotherapeutic agents is most likely to have caused the second malignancy?
A. Asparaginase.
B. Cyclophosphamide.
C. Doxorubicin.
D. Vincristine.
E. 6-Mercaptopurine.
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Question 7
You are asked to consult on the aerial transfer of a sick infant with bronchiolitis. By what amount will you
expect the alveolar oxygen to drop when the plane is pressurised at an altitude of 5000 feet?
(The atmospheric pressure at 5000 feet is 647 mmHg. The carbon dioxide level is 45 mmHg, the respiratory
quotient is 0.8 and the water vapour pressure is 47 mmHg. These values remain constant. The flight will
begin at sea level where the atmospheric pressure is 760 mmHg.)
A. 5-10 mmHg.
B. 11-15 mmHg.
C. 16-20 mmHg.
D. 21-25 mmHg.
E. 26-30 mmHg.
Question 8
I:1 and his wife I:2 take part in a Tay-Sachs carrier screening program and are both shown to be carriers. Their
children have not been tested. The frequency of the heterozygous state is 1 in 30 in the particular population from
which all these individuals in this pedigree are drawn.
The risk that the foetus III:1 will be affected with Tay-Sachs disease is closest to:
A. 1 in 240.
B. 1 in 180.
C. 1 in 120.
D. 1 in 60.
E. 1 in 30.
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Question 9
Which one of the following groups of physiological parameters best describes vaso-vagal syncope?
BP HR SVR
A. low low high
B. low low normal
C. low low low
D. normal low low
E. low normal low
Note:
BP = blood pressure
HR = heart rate
SVR = systemic vascular resistance
Question 10
Which associated feature of attention deficit hyperactivity disorder at presentation is most likely to predict a poorer
response to stimulant medication treatment?
A. Age greater than 10 years.
B. Co-existing anxiety disorder.
C. Female sex.
D. Prominent aggression.
E. Severe inattentiveness.
Question 11
Which one of the following partial pressures of arterial oxygen would you expect from a normal one-year-old
breathing 60% oxygen at sea level?
A. 290 mmHg.
B. 390 mmHg.
C. 450 mmHg.
D. 520 mmHg.
E. 570 mmHg.
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Question 12
The principle mechanism which ensures excretion of excess sodium when the dietary intake of salt is high
involves:
A. atrial natriuretic factor.
B. glomerular filtration.
C. Na-K ATPase.
D. renin-angiotensin pathway.
E. tubulo-glomerular feedback.
Question 13
The laboratory telephones you with the following test results of blood taken from a 16-year-old male student
who had requested hepatitis B screening.
HBsAg (surface antigen) negative
Anti-HBs (surface antibody) positive
Anti-HBc (core antibody) positive
The most likely explanation for these results is:
A. acute hepatitis B infection.
B. chronic hepatitis B infection.
C. hepatitis D superinfection.
D. past hepatitis B vaccination.
E. previous hepatitis B infection.
Question 14
A clear preference for using the left hand at 12 months of age is most likely a manifestation of which one of the
following?
A. Brachial plexus injury.
B. Cerebral palsy.
C. Family history of left handedness.
D. Normal variation.
E. Osteomyelitis.
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Question 15
The anticoagulant effect of the coumarin derivative warfarin is most likely to be enhanced by which one of the
following?
A. Carbamazepine.
B. Erythromycin.
C. Oral contraceptive.
D. Phenytoin.
E. Vitamin K.
Question 16
Which one of the following is most important in recovery from acute bronchiolitis due to respiratory syncytial virus?
A. IgG antibody production.
B. IgM antibody production.
C. Interferon.
D. Natural killer cells.
E. T lymphocytes.
Question 17
During a phase 1 clinical trial, a new drug, neobantrine, radio-labelled with tritium, was administered orally to
volunteer subjects. Recovery of radioactivity over the next 48 hours was as follows:
faeces 4%, all as neobantrine glucuronide
urine 80%, as neobantrine glucuronide
12%, as unchanged neobantrine.
An earlier study had examined plasma concentrations of unchanged neobantrine after intravenous administration,
compared to oral administration. The analysis of the concentration versus time for oral and IV administration,
which was expressed as area under the curve (AUC) was as follows:
AUC oral
------------ = 0.2
AUC IV
The best description of the metabolic pathways of neobantrine, based on this study is:
A. good absorption from the gut, extensive first pass effect, clearance primarily by hepatic conjugation.
B. good absorption from the gut, little first pass effect, clearance primarily by hepatic conjugation.
C. good absorption from the gut, little first pass effect, clearance primarily by renal excretion.
D. poor absorption from the gut, extensive first pass effect, clearance primarily by hepatic conjugation.
E. poor absorption from the gut, little first pass effect, clearance primarily by renal excretion.
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Question 18
A Weschler Intelligence Scale for Children (WISC) assesses general intelligence and its contributing factors.
Which one of the following requires an additional test in order to be adequately measured?
A. Freedom from distractibility.
B. Perceptual organisation.
C. Reading ability.
D. Speed of information processing.
E. Verbal comprehension.
Question 19
A frameshift mutation is best described as a:
A. missing base or set of bases which results in a premature stop codon.
B. mutation which alters the codons downstream of its location.
C. mutation which alters the normal splicing of an intron.
D. sequence alteration which occurs at or near regulatory sequences in the 5' untranslated region of the
gene.
E. single base change which alters that codon to one which now reads stop.
Question 20
The cavernous sinus contains all of the following nerves except:
A. abducens.
B. facial.
C. oculomotor.
D. trigeminal.
E. trochlear.
Question 21
A patient with pancreatic insufficiency refuses to take enzyme replacement and later presents with renal calculi.
Increased excretion of which one of the following is the most likely cause of calculus formation?
A. Bicarbonate.
B. Cysteine.
C. Oxalate.
D. Phosphate.
E. Urate.
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Question 22
In a four-year-old child, marked dilatation of the pulmonary artery would be most likely to occur in which one of the
following?
A. Atrial septal defect.
B. Bilateral pulmonary artery branch stenosis.
C. Idiopathic arterial calcification.
D. Mild pulmonary valve stenosis.
E. Severe pulmonary valve stenosis.
Question 23
A child suspected of having developmental delay, repeatedly and purposefully uncovers a toy you have covered
with a piece of cloth. He seems interested in the toy and is aware that it is under the cloth even when he does not
see you cover it.
It is generally accepted that normal children should have acquired this skill by age:
A. 9 months.
B. 12 months.
C. 15 months.
D. 18 months.
E. 21 months.
Question 24
For which one of the following antiepileptic drugs is the measurement of plasma levels of most value in clinical
management?
A. Carbamazepine.
B. Lamotrigine.
C. Phenytoin.
D. Sodium valproate.
E. Vigabatrin.
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Question 25
A Streptococcus pneumoniae isolate with high-level resistance to penicillin (MIC>1 mg/L) is most likely to be
sensitive to which one of the following antibiotics?
A. Cefotaxime.
B. Chloramphenicol.
C. Ciprofloxacin.
D. Erythromycin.
E. Vancomycin.
Question 26
Which one of the following is not a recognised cause of nephrotic syndrome?
A. Autosomal dominant thin membrane disease.
B. Focal segmental glomerulosclerosis.
C. Membranoproliferative glomerulonephritis.
D. Membranous glomerulonephritis.
E. Post-infectious glomerulonephritis.
Question 27
For which one of the following organisms is infection most likely to confer lasting protection against reinfection?
A. Influenza A virus.
B. Measles virus.
C. Respiratory syncytial virus.
D. Parainfluenza virus.
E. Rotavirus.
Question 28
The most common cystic fibrosis gene mutation is a result of:
A. codon deletion.
B. duplication.
C. frame shift.
D. inversion.
E. splice-site mutation.
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Question 29
The relative potency of the glucocorticoid activity of betamethasone compared to prednisolone is closest to which
one of the following ratios?
A. 3:1.
B. 6:1.
C. 10:1.
D. 15:1.
E. 25:1.
Question 30
Which one of the following primary immunodeficiency disorders is most likely to be associated with anaphylaxis to
blood products?
A. Ataxia-Telangiectasia.
B. Common variable immunodeficiency.
C. IgG subclass deficiency.
D. Selective IgA deficiency.
E. X-linked agammaglobulinaemia.
Question 31
What is the risk that IV:1 in the pedigree shown is affected, if the genetic disorder depicted has a population
frequency of 1 in 20,000?
A. 1 in 2.
B. 1 in 4.
C. 1 in 5,000.
D. 1 in 10,000.
E. 1 in 20,000.
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Question 32
A 13-year-old boy has been diagnosed with insulin dependent diabetes for eight years and coeliac disease for four
years. When he is reviewed, he complains of mild lethargy. He is noted to be more pigmented than normal.
Which one of the following tests would be most useful in diagnosing adrenal insufficiency in this situation?
A. Adrenal autoantibodies.
B. Plasma ACTH (adrenocorticotrophic hormone).
C. Plasma alpha MSH (melanocyte stimulating hormone).
D. Plasma cortisol.
E. Urinary free cortisol.
Question 33
A previously well eight-year-old boy presents to his paediatrician with recent onset left-sided facial weakness.
Which one of the following would suggest that this is not a Bell's palsy?
A. Frontalis weakness.
B. Hyperacusis.
C. Impaired lacrimation.
D. Loss of taste to the posterior third of the tongue.
E. Subjective sensory disturbance of the face.
Question 34
The total number of hospital admissions with asthma in Australia and New Zealand has increased.
What is the one most important factor in this rise?
A. Changes in diagnostic categorisation.
B. Decreased threshold for admission.
C. Greater awareness by general practitioners.
D. Increased use of inhaled beta agonists.
E. Increased incidence of asthma.
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Question 35
Which one of the following is the mechanism for penicillin resistance ofStreptococcus pneumoniae?
A. Altered penicillin binding proteins.
B. Beta-lactamase production.
C. Plasmid-mediated resistance.
D. Ribosomal inhibition of protein synthesis.
E. Transposon-mediated resistance.
Question 36
A three-year-old boy presents with acute hepatocellular injury secondary to viral hepatitis.
Which one of the following tests most accurately assesses his current liver synthetic function?
A. Alanine aminotransferase.
B. Ammonia.
C. Conjugated bilirubin level.
D. Gamma glutamyltransferase.
E. Prothrombin time following parenteral vitamin K.
Question 37
A newborn infant is found to be hypothyroid on a new born screening test.
Which one of the following is the most likely cause?
A. Defective thyroglobulin iodination.
B. Deficiency of thyroid-stimulating hormone.
C. Impaired foetal iodine transport.
D. Maternal autoantibodies.
E. Thyroid dysgenesis.
Question 38
Which one of the following psychotropic drugs has the greatest alpha-adrenergic agonist effect?
A. Clomipramine.
B. Clonidine.
C. Fluoxetine.
D. Haloperidol.
E. Thioridazine.
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Question 39
What is the most important mechanism by which the drug omeprazole exerts its effect on the gastric mucosa in
the treatment of peptic ulcer disease?
A. Binds to the prostaglandin E2 receptor.
B. Binds to the hydrogen potassium ATPase pump.
C. Blocks H1 receptors.
D. Blocks H2 receptors.
E. Blocks secretion of gastric intrinsic factor.
Question 40
The variance for height (or weight) in a population may be expressed in percentiles or standard deviation scores.
One standard deviation below the population mean (-1 SD) approximates which one of the following percentiles?
A. 3rd.
B. 10th.
C. 15th.
D. 25th.
E. 33rd.
Question 41
Which of the following immunoglobulins fix complement when they bind to antigen?
A. Only IgG.
B. IgG and IgA.
C. IgG, IgA and IgM.
D. IgG and IgM.
E. Only IgM.
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Question 42
A two-year-old boy with Duchenne muscular dystrophy (DMD) has an identified mutation within the DMD gene.
Although his mother does not have this mutation in the blood and there is no clinical or biochemical evidence to
suggest that she is a carrier, there is still a low recurrence risk among his male siblings.
Which one of the following mechanisms is the most likely explanation for these recurrences?
A. An unstable triplet repeat mutation.
B. Autosomal recessive inheritance.
C. Gonadal mosaicism.
D. Incomplete penetrance.
E. Polygenic inheritance.
Question 43
Which one of the following is predominantly excreted unchanged in the urine?
A. Carbamazepine.
B. Gabapentin.
C. Lamotrigine.
D. Phenytoin.
E. Sodium valproate.
Question 44
When assessing the response of small airways to bronchodilators, the best test is:
A. forced expiratory volume in 1 second/forced vital capacity (FEV1/FVC) ratio.
B. functional residual capacity (FRC).
C. inspiratory capacity.
D. iso volume forced expiratory flow (FEF25-75).
E. peak flow.
Question 45
Which one of the following target autoantigens is least likely to be associated with type 1 (insulin-dependent)
diabetes mellitus?
A. Double-stranded DNA.
B. Endomysial antigen.
C. Glutamic acid decarboxylase.
D. Thyroid peroxidase.
E. 21-hydroxylase.
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Question 46
During her first pregnancy, a woman develops chickenpox at 16 weeks gestation.
Which one of the following represents the closest approximation to the risk that the baby will develop congenital
varicella syndrome?
A. 0.01%.
B. 2%.
C. 15%.
D. 50%.
E. 80%.
Question 47
What is the most important pathophysiological basis of gastroesophageal reflux?
A. Impaired oesophageal clearance of acid.
B. Impairment of resting lower oesophageal sphincter tone.
C. Increased gastric acid production.
D. The presence of a hiatus hernia.
E. Transient relaxations of the lower oesophageal sphincter.
Question 48
Drug X has linear (first order) pharmacokinetics and a narrow therapeutic index and is cleared by both renal
filtration (80%) and hepatic metabolism (20%). The standard daily dose required by an individual with normal
renal and hepatic function to achieve a standard therapeutic response is 100 mg.
Which one of the following is the best estimate of the daily maintenance dose that would be required in a person
with a creatinine clearance one quarter of normal?
A. 20 mg.
B. 25 mg.
C. 30 mg.
D. 35 mg.
E. 40 mg.
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Question 49
The reabsorption of most bicarbonate (HCO3) from the glomerular ultrafiltrate is by:
A. diffusion of carbon dioxide (CO2).
B. intercellular transport.
C. pinocytosis.
D. primary active transport.
E. transcellular aquapores.
Question 50
A two-year-old girl presents with failure to thrive and emaciation. You suspect that a brain tumour may be the
cause.
The most likely position for a brain tumour to give rise to these symptoms would be in the:
A. anterior portion of the hypothalamus.
B. cerebellum.
C. corpus callosum.
D. pons of the brainstem.
E. posterior part of the fourth ventricle.
Question 51
Four cytokines produced by T cells are listed:
interleukin 2 (IL-2)
interleukin 4 (IL-4)
interleukin 5 (IL-5)
interferon gamma (IFN)
Which one of the following combinations would best reflect a Th2 type T cell immune response?
A. IL-2, IFN.
B. IL-2, IL-4.
C. IL-4, IFN.
D. IL-4, IL-5.
E. IL-5, IFN.
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Question 52
The plasma half-life of nitric oxide is extremely short.
This is primarily due to:
A. binding of nitric oxide to haemoglobin.
B. binding of nitric oxide to plasma albumin.
C. metabolic degradation of nitric oxide within vascular muscle fibres.
D. uptake of nitric oxide by alveolar macrophages.
E. uptake of nitric oxide by alveolar type II cells.
Question 53
Which one of the following is the most common biochemical association with obesity?
A. Mutation of the beta three adrenergic receptor.
B. Mutation of the insulin receptor.
C. Raised blood glucose.
D. Raised serum c peptide.
E. Reduced serum leptin.
Question 54
Which one of the following drugs crosses the placenta to a negligible degree?
A. Captopril.
B. Digoxin.
C. Pancuronium.
D. Sodium valproate.
E. Warfarin.
Question 55
Examination of the synovial fluid from a subject with rheumatoid arthritis reveals many polymorphs and the
following factors. Which one of these is likely to be predominantly responsible for attracting polymorphs into the
synovial fluid?
A. Complement C4.
B. Complement C5a.
C. Interleukin 1.
D. Interleukin 2 receptors.
E. Tumour necrosis factor.
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Question 56
The most common dietary factor associated with iron deficiency in infancy is:
A. exclusive breast feeding with introduction of solids later than six months.
B. exclusive formula feeding with non iron supplemented formula.
C. lack of ascorbic acid supplementation.
D. introduction of cereals prior to four months.
E. introduction of unmodified cows milk prior to eight months.
Question 57
If the full immunisation schedule has been properly administered to a two-year-old, which one of the following
gives the least effective protection?
A. Diphtheria.
B Haemophilus influenzae type b.
C. Measles.
D. Pertussis.
E. Rubella.
Question 58
Which one of the following is an endothelium-derived vasodilator?
A. Angiotensin II.
B. Endothelin 1.
C. Prostacyclin.
D. Prostaglandin H2.
E. Thromboxane A2.
Question 59
Deficiency of which one of the following components of neutrophils is most likely to result in the development of
granulomas?
A. Actin.
B. Cell adhesion molecules.
C. Fc receptors.
D. Myeloperoxidase.
E. NADPH oxidase.
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Question 60
A four-year-old girl is thought to have Prader-Willi syndrome. To confirm the clinical diagnosis, DNA samples from
the child and her parents have been studied. The results shown below are obtained after probing a Southern blot
with a DNA probe located within the region of chromosome 15 that is commonly deleted in this syndrome. This
probe detects a restriction fragment length polymorphism with two alleles in men and women in the normal
population.
What is the molecular basis of Prader-Willi syndrome in this girl?
A. Deletion of the maternally-derived chromosome 15.
B. Disomy of both parental chromosomes.
C. Partial deletion of both parental chromosomes 15.
D. Uniparental disomy of the maternally-derived chromosome 15.
E. Uniparental disomy of the paternally-derived chromosome 15.
Question 61
The mean pulmonary artery (capillary) wedge pressure most closely approximates which one of the following
pressures?
A. Left atrial mean.
B. Pulmonary artery diastolic.
C. Pulmonary vein wedge mean.
D. Right atrial mean.
E. Right ventricular end-diastolic.
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Question 62
Which one of the following problems is not associated with craniopharyngioma?
A. Bitemporal hemianopia.
B. Loss of visual acuity.
C. Macula degeneration.
D. Optic atrophy.
E. Papilloedema.
Question 63
A one year old presents with gastroenteritis and mild dehydration (5%). Electrolytes are obtained:
sodium 129 mmol/L [135-145]
potassium 4.0 mmol/L [3.5-5.5]chloride 98 mmol/L [98-110]
bicarbonate 22 mmol/L [21-26]
urea 5.0 mmol/L [1.3-6.6]
What would be the most likely physiological response to this disturbance of salt and water homeostasis?
Aldosterone Atrial Natriuretic
Peptide
Arginine Vasopressin Angiotensin II
A. Decreased Decreased Increased
B. Increased Decreased Decreased
C. Increased Decreased Increased
D. Decreased Decreased Increased
E. Increased Increased Increased
Question 64
The cell type playing the predominant role in the late inflammatory phase of an IgE mediated allergic response is
the:
A. basophil.
B. eosinophil.
C. mast cell.
D. monocyte.
E. neutrophil.
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Question 68
The autoantibody most specific for systemic lupus erythematosus is:
A. antihistone.
B. antinuclear.
C. anti-ribonucleoprotein.
D. anti-Ro (SS-A).
E. anti-Sm.
Question 69
In congestive cardiac failure, which one of the following factors is most important in the pathogenesis of peripheral
oedema?
A. Hypoalbuminaemia.
B. Impaired lymphatic drainage via thoracic duct.
C. Increased antidiuretic hormone production.
D. Increased capillary permeability.
E. Sodium retention.
Question 70
The predominant cause of nodule formation in a positive tuberculin skin test reaction is infiltration with:
A. CD4+
T lymphocytes.
B. CD8+
T lymphocytes.
C. eosinophils.
D. macrophages.
E. neutrophils.
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1999 FRACP Written Examination
Paediatrics & Child Health
Paper 1 Medical Sciences
Answers
1. D 25. E 49. A
2. D 26. A 50. A
3. C 27. B 51. D
4. B 28. A 52. A
5. E 29. B 53. D
6. B 30. D 54. C
7. D 31. A 55. B
8. B 32. B 56. E
9. C 33. D 57. D
10. B 34. E 58. C
11. B 35. A 59. E
12. A 36. E 60. D
13. E 37. E 61. A
14. B 38. B 62. C
15. B 39. B 63. C
16. E 40. C 64. B
17. A 41. D 65. C
18. C 42. C 66. B
19. B 43. B 67. D
20. B 44. D 68. E
21. C 45. A 69. E
22. D 46. B 70. D
23. C 47. E
24. D 48. E