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FC Paed(SA) Part I THE COLLEGES OF MEDICINE OF SOUTH AFRICA

Incorporated Association not for gain Reg No 1955/000003/08

Part I Examination for the Fellowship of the College of Paediatricians of South Africa 25 March 2014 Paper 2 (3 hours) ___________________________________________________________________________________________ Instructions 1 Answer each of the following SIX (6) questions in separate books. All questions are to be answered. 2 Each question is worth 30 marks you should not spend more than 30 minutes per question. 3 The aim is to assess your ability to express knowledge concisely and precisely. 4 You may answer the questions in Afrikaans, if you wish.
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-2Question 1 Lindiwe, a 3-year-old girl, presents to medical emergency with a history of being unable to walk since waking from her afternoon rest. She had an episode of mild gastroenteritis 2 weeks previously. On examination you find her to be generally floppy with markedly reduced tone in her lower limbs and absent deep tendon reflexes. She has a weak cry and some head-lag. You suspect GuillainBarr Syndrome (GBS). a) List 3 (THREE) differential diagnoses other than GBS. Polymorphonuclear cells Lymphocytes Protein 0 per high power field. 4 per high power field. 1.6mg/L. (3)

Her lumbar puncture reveals

b) c) d)

Describe the various structures traversed when doing a lumbar puncture. (7) Briefly discuss the pathophysiology of Guillain-Barr syndrome. (5) List 2 (TWO) other investigations which might help confirm your diagnosis and discuss each possible result. (4)

Intravenous immunoglobulin (IVIG) is administered to Lindiwe. e) f) g) h) What does IVIG consist of? Briefly discuss the immune modulating action of IVIG. List 3 (THREE) long term complications of GBS that you would need to manage. Describe 2 (TWO) necessary public health measures in this case. (2) (4) (3) (2) [30]

Question 2 A 900 g infant is delivered vaginally at 28 weeks of gestational age. a) b) Describe 4 (FOUR) mechanisms of heat loss in this neonate. (4) List 2 (TWO) ways in which hypothermia can be prevented immediately after delivery. (2)

The infant develops respiratory distress with sternal recession. The oxygen saturation is 75% in room air. After nasal prong oxygen is started it improves to 86%. The CXR shows small lung volumes with a ground glass appearance. c) d) e) What is the most likely diagnosis? Motivate your answer. (2) Describe the pathophysiology of this condition. (6) List 3 (THREE) further investigations that may confirm or help exclude the likely diagnosis. (3)

The infant is transferred to NICU for ventilation. A 5% glucose intravenous solution is commenced at a rate of 100 ml/kg/day. f) g) Calculate the amount of glucose the baby is receiving. How much glucose does a premature baby require? (2) (1)

PTO/Page 3 Question 2 h)

-3The electrolyte results at 8 hours are Na+ 152 mmol/l. K+ 5.2 mmol/l. Cl120 mmol/l. HCO318 mmol/l. Urea 7 mmol/l. Creatinine 80 mol/l. h) i) j) Identify 3 (THREE) abnormalities and offer a possible explanation for each. Describe how you will manage any two of these abnormalities. Briefly discuss how the renal function of a neonate differs from that of an adult. (3) (2) (5) [30]

Question 3 A 6-month-old child is referred to you with delayed development. Trisomy 21 (Down syndrome) is diagnosed clinically. His mother is 25-years-old. a) List 3 (THREE) diagnostic tests available to confirm this condition and indicate which one you will order in this patient. (4)

On cardiac ultrasound an endocardial cushion defect is diagnosed. b) c) Describe the embryology of this defect. Describe 2 (TWO) ECG abnormalities expected in this patient. (6) (2)

An unbalanced translocation is found in the child involving the long arms of chromosomes 14 and 21. d) e) f) Explain what is meant by an unbalanced translocation. (2) List 2 (TWO) other genetic variations that can occur in Down syndrome and explain what each type means. (4) Describe how you will counsel the mother with regard to future child bearing. (3)

As part of the routine diagnostic testing auditory brainstem response (ABR) audiometry is done. Bilateral mild hearing loss is found. g) Briefly describe the physiology of an auditory brainstem response. (4)

The prevalence of congenital hypothyroidism in Down syndrome is twenty-eight times greater than in the general population. You decide to do test the childs thyroid function. h) i) Which specific tests will you order and why? Which drug will you use for replacement should it be necessary? (4) (1) [30]

PTO/Page 4 Question 4

-4Question 4 Sachin, a 12-month-old child of Indian descent, arrives in casualty with a 6 week history of irritability, lethargy and poor feeding. There is no jaundice or hepatosplenomegaly. The haemoglobin level is 6 g/dl and the platelet count is 100 x 109/l. The white cell count is normal. a) b) Is this child anaemic? Explain. Describe the changes in erythropoiesis from birth to 1-year-of age with regard to i) The types of haemoglobin and the globin chains that are involved. ii) The MCV in relation to the type of haemoglobin. Indicate 3 (THREE) likely differential diagnosis for the anaemia in this child. Explain the terms RDW, MCHC and MCV found on the full blood count report. (2) (4) (2) (3) (3)

c) d)

The child is found to have developmental delay and the MCV is 110fl. e) f) g) h) List 4 (FOUR) differential diagnoses of a macrocytic anaemia in infancy and childhood. (4) What features on the full blood count and smear will lead you to the diagnosis of a megaloblastic anaemia? (3) What are the causes of megaloblastic anaemia in infancy and childhood? (3) What is your approach to investigating megaloblastic anaemia in infancy and childhood? (6) [30]

Question 5 Thabo, a two-year-old boy presents to a local clinic with vomiting and diarrhoea for two days. He has been receiving home-made sugar salt solution (SSS). a) b) Describe how home-made SSS should be prepared. Why are the following fluids inappropriate for oral rehydration i) Sweetened fruit drinks? ii) Soup? With reference to the constitution of SSS, discuss water absorption in the intestine. (2)

c)

(2) (5)

He is assessed as having severe dehydration and is provided with Reduced Osmolarity Oral Rehydration Solution (ORS). d) e) f) g) What does Reduced Osmolarity ORS contain? (3) How is this different from standard ORS? (2) Name one advantage of Reduced Osmolarity ORS over standard ORS. (1) How does ReSoMal (Rehydration Solution for Malnourished children) differ from the ORS described above? (2)

You are unsure whether Thabo will benefit from receiving zinc supplementation and decide to research the topic. You find the following figure in a 2008 Cochrane Review of the subject.

PTO/Page 5 Question 5 h)

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Based on this analysis answer the following h) i) j) k) l) m) What type of analysis has been performed? (1) What is this format of data presentation called? (1) Is zinc supplementation better than placebo in children aged < 6 months? Explain your answer. (3) The Total row at the bottom indicates a mean difference of -12.27. What does this mean? (2) The Total row further indicates [-23.02, -1.52]. What does this mean? (2) Would you recommend routine zinc supplementation to all children with acute diarrhoea, based on this study? Justify your response. (4) [30]

PTO/Page 6 Question 6

-6Question 6 Julius, an 18-month-old boy, presents to a community health clinic with a short history of fever, loss of appetite and poor feeding. He is febrile, jaundiced and has hepatosplenomegaly. a) b) List 4 (FOUR) side room investigations that may help in diagnosis and management. Explain how each will help. (4) List 3 (THREE) beneficial effects of fever. (3)

Shortly after presentation, Julius develops tachypnoea and has repeated left-sided seizures. You consider a diagnosis of malaria. c) d) Briefly discuss the laboratory diagnosis of malaria. Identify 3 (THREE) features indicating SEVERE malaria in this child. (4) (3)

Juliuss mother mentions that they have been to a holiday resort in Mozambique 9 days previously. e) Briefly discuss malaria prophylaxis in infants and children with regard to i) General advice. ii) Medication.

(4) (5)

Immediate notification [within 24-hours] after diagnosis by the health care professional through telephone or fax to the designated district or provincial health officer is necessary for some diseases.
f)

Is malaria one of these conditions?

(1)

Two weeks after discharge and apparent recovery Julius again has fever.
g) h)

Explain the difference between relapses and recrudescence of malaria. List 2 (TWO) malaria species that cause relapses.

(4) (2) [30]

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FC Paed(SA) Part I

THE COLLEGES OF MEDICINE OF SOUTH AFRICA


Incorporated Association not for gain Reg No 1955/000003/08

Part I Examination for the Fellowship of the College of Paediatricians of South Africa 26 March 2014 Paper 3 Instructions 1 Answer each of the following FOUR (4) questions in separate books. All questions are to be answered. Each question has 4 sub-questions. There are 16 sub-questions in total. Answers to each sub-question should be brief and to the point. Each sub-question is worth 10 marks. The whole paper is worth 160 marks. You should not spend more than 11 minutes per sub-question. These are short note type questions. The aim is to assess your ability to express knowledge concisely. You may answer the questions in Afrikaans, if you wish. (3 hours)

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-81 Write short notes on a) C-reactive protein and its usefulness in paediatric practice. b) Enterobacteriaceae with reference to i) Microbiological characteristics. ii) Important genera in the family. iii) Mechanisms of antibiotic resistance. c) d)

(10)

(4) (3) (3) (10) Iron metabolism, including its distribution in the body, absorption, excretion, plasma transport, and the role of hepcidin. (10) Phase 1, 2, 3 and 4 clinical trials. (10) [40]

Write short notes on a) The endocrine causes of short stature in children. b) The aetiological factors in childhood tumours. c) The long-term side-effects of oral corticosteroid use in children. d) The pathophysiology of disseminated intravascular coagulopathy (DIC).

(10) (10) (10) (10) [40]

Write short notes on a) The aetiology and pathophysiology of acute urticaria. b) Anatomy of portal systemic anastomoses in portal hypertension. c) The diagnostic value of tests performed on pleural fluid. d) The pulmonary effects of drowning.

(10) (10) (10) (10) [40]

Write short notes on a) Obstructive sleep apnoea in children with reference to the following i) Definition. ii) Pathophysiology of snoring. iii) Factors predisposing children to obstructive sleep apnoea. b) Rotavirus vaccines with reference to the following i) Available vaccine types. ii) Vaccine safety. iii) Contra-indications. iv) Schedule. The causes and clinical manifestations of hyperkalaemia in children. The causes and metabolic consequences of persistent vomiting.

(4) (3) (3) (10) (4) (2) (3) (1) (10) (10) (10) [40]

c) d)