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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 27 August 2013 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 4 The aim is to assess your ability to express knowledge concisely and precisely. You may answer the questions in Afrikaans, if you wish.

g) h) Which drug will you use for treatment? Briefly explain the mechanism of antiviral action of this drug. (4) The mother’s HIV Elisa is confirmed positive.-2  Question 1 You admit a 20-year-old woman who gave birth to a healthy 3. She mentions that the rash started three days before. k) l) m) List two antimicrobial substances found in breast milk. (1) List two pathogenetic mechanisms for thrombocytopenia in the neonate with two examples of diseases in each group. (4) (1) (3) [30] . and indicate their function. d) e) f) Explain the rationale behind this decision. On examining the mother you find chicken-pox lesions. (3) (1) (3) Despite receiving the VZIG the baby develops chickenpox. (1) (2) On the blood count during therapy the platelet count is low. She enquires about stopping breastfeeding and the use of banked human breast milk. How is donor breast milk treated before it is dispensed? List three other infections that human milk donors should be screened for. a) b) c) Which virus causes chickenpox? (1) To which family of viruses does it belong? (1) Should the fetus have been infected with chickenpox before 20 weeks of gestation. i) j) Define thrombocytopenia in the newborn. She is concerned about transmitting HIV and or other diseases to her child. What type of immunisation does this constitute? List 3 other infections where this type of immunisation is used.5 kg boy at home an hour ago. list five clinical features. (5) As part of your management you administer varicella-zoster immune globulin (VZIG) to the neonate.

who is now 4-years-old. However before the appointment date you are again called to the emergency centre to see Promise. h) i) j) What is this condition and explain its pathophysiology? Name 3 other “painful crises” associated with SCD and how do they present? Hydroxyurea is sometimes used in the management of SCD. (6) Promise’s mother is 5 months pregnant.-3  Question 2 A 3-year-old girl. l) To what is he referring? (1) [30] . (2) The family are lost to follow-up. Promise has never travelled to the Congo. This child is 8-months-old and presents with swollen and apparently painful fingers bilaterally. On examination she is pale with mild jaundice and clinically has features of heart failure. Her haemoglobin is 4g/dL. How does this drug work? (2) (3) (2) When the younger sibling is ready for discharge you make appointments for both children at your outpatient clinic. (3) How would the diagnosis of SCD be confirmed in the laboratory? Briefly explain the test done. g) What advice would you give regarding the risk for the newborn and testing for SCD? Explain your answer. You suspect she may have sickle cell disease (SCD). (1) You meet her father during this admission. You confirm the diagnosis of sickle cell disease. is brought into the Emergency Department with a 2 day history of shortness of breath and tiredness. She has also had a fever for 3 days. Promise is stabilised and appropriately managed. a) b) c) d) e) Briefly explain the basic aetiology and pathophysiology of this condition (SCD). He says that he has heard from a relative who is living in France that there is now a cure for SCD and he is requesting this for his children. providing an explanation for each. She presents with an acute history of sudden weakness of her right arm and leg and is unable to speak. k) Explain what has most likely occurred. (3) Briefly outline your initial management. Promise. You struggle to get any further history as her mother is Congolese and only speaks French. You recognise the mother when you are called to evaluate a child in the emergency centre. (3) Why are children with SCD often stunted when older? (1) During her hospital stay. She is now ready for discharge f) Outline your discharge plan including medications. (3) Give a differential diagnosis for what may have precipitated this current presentation.

(2) (6) Baby Booi appears pale and lethargic and you are concerned that he has developed nosocomial sepsis. Discuss the pharmacology and adverse effects of one of these drugs. born at 29 weeks gestation with a birth weight of 1170 g. (6) (4) His blood culture grows an extended spectrum beta-lactamase producing Klebsiella pneumonia. e) f) Discuss possible pathophysiological mechanisms for this result. You order some blood tests. g) h) Describe the microbiology of Klebsiella. By day 4 of life he copes well on room air while oral feeds are progressively increased. Explain the use and interpretation of the full blood count in neonatal sepsis. (4) (4) Baby Booi’s serum sodium is 128 mmol/L. a) b) Name 2 drugs commonly used to treat apnoea in the neonate. You are called to see him on day 8 of life as he is now having recurrent apnoea.-4  Question 3 Baby Booi is 8-days old. Outline your approach to the management of this. His clinical and chest x-ray suggest respiratory distress syndrome. He is treated with nasal continuous positive airway pressure (CPAP) for 2 days. c) d) Discuss the synthesis and function of C-reactive protein in sepsis. (2) What are beta-lactamases and what is the implication of identifying an extended spectrum beta-lactamase producing organism? (2) [30] .

g) h) i) What is the most likely complication now? Briefly describe how Karabo's cardiac condition predisposed her to this. A chest x-ray is performed and shows cardiomegaly with a cardiothoracic ratio of 70% and increased pulmonary vascularity. Her liver is palpable 4cm below the costal margin. (1) (1) (1) b) (1) (1) (1) (1) c) d) e) f) What is the most likely cardiac lesion present? (1) Explain why Karabo was asymptomatic at or immediately after birth. Karabo has a respiratory rate of 60/min and a heart rate of 150 beats per minute. over the past month. (1) (2) (10) [30] . She has subcostal and intercostal recessions. She was born at term with a birth weight of 3 kg. Pulses are all palpable with increased volume. Briefly describe the mechanism for each of these findings i) High volume pulses. The trachea is central. iii) Absent pulse. Splenomegaly is present. (2) Which chamber(s) of the heart are likely to be enlarged in this child? Explain. She is pale and her temperature is 39°C. She has difficulty breathing immediately after feeding and cannot complete feeds. she has not been gaining weight well and has trouble feeding. (2) Briefly describe the neuro-hormonal mechanisms involved in her cardiac failure. iii) Loud pulmonary component of the second heart sound. There were no perinatal or neonatal problems. A mid-diastolic murmur is audible at the apex. Her blood pressure is 80/25mmHg. Karabo is seen two weeks later with fever. Discuss the pathophysiological basis of her i) Fever. Her oxygen saturation is 99% while breathing room air. Her right upper limb pulse cannot be palpated and her urine tests positive for blood. v) Haematuria. ii) Splenomegal. A diagram may be used. ii) Left parasternal and epigastric pulsations. iii) Mid-diastolic murmur at the apex.-5  Question 4 Karabo is a two-month-old girl who is brought to hospital as her mothers concerned that. The cardiac apex is displaced lateral to the mid-clavicular line and is hyperdynamic. The pulmonary component of the second heart sound is loud and a loud systolic murmur is auscultated in the left infra-clavicular area. A prominent parasternal as well as epigastric pulsation is palpated. a) Interpret the significance of the following clinical findings i) Displaced apex beat. ii) Systolic murmur in the left infra-clavicular area. iv) Pallor. iv) Increased pulmonary vascularity on chest x-ray.

explaining the role of the above organisms. She is noted to be pale. (3) What do you expect to find on Ziyanda’s peripheral blood smear? (2) Which two organisms are most likely to have caused the diarrhoea? (2) Outline the pathogenesis of this condition. Her serum creatinine is 200 µmol/L. f) g) Explain the pathophysiology of Ziyanda’s hypertension. The previous week she had an episode of bloody diarrhoea after visiting her uncle’s farm a few days before. (3) Ziyanda’s blood pressure is 160/110mmHg. her haemoglobin is 6 g/dL and her platelet count is 45 000/mm3 a) b) c) d) e) List three differential diagnoses. a 5-year-old girl presents to the emergency department with a history of oliguria and fatigue. (10) List three other causes (atypical variants) of this condition and explain briefly their underlying pathophysiology. (5) [30] . m) Outline the underlying principles of peritoneal dialysis.-6  Question 5 Ziyanda. Outline the principles of managing her hypertension. (2) (3) Ziyanda is started on peritoneal dialysis. Urine dipstick is positive for blood and protein.

c) d) e) What are the three most likely causes of Peter’s meningitis? List three other less common causes of meningitis that you could consider.9°C. His blood pressure is 70/52mmHg. a 6-year-old boy. Discuss the pathophysiology of septic shock. is seen at the rooms of a family physician. (3) (3) (6) Peter’s parents indicate that he has received all his vaccinations and cannot understand why he has developed meningitis. h) i) Discuss the mechanism of action of cephalosporins. He has neck stiffness and is fully conscious. He has vomited once a few hours ago and has fever and headache. b) List four causes of neck stiffness in a child.-7  Question 6 Peter. What explanation would you offer the parents to address their concern? (2) (2) The doctor inserts a peripheral venous line. He has only had minor illnesses in the past and has never been admitted to hospital. gives a fluid bolus and commences a cephalosporin. He has not travelled outside of South Africa. (3) (3) [30] . f) g) Which vaccines are available to prevent meningitis? Include timing of the vaccines. a) What are the advantages of pyrexia in a child with an infection? (4) The doctor examines him and confirms a fever of 38. Outline three adverse effects of cephalosporins. (4) A diagnosis of meningitis is made.

You should not spend more than 11 minutes per sub-question. if you wish. The whole paper is worth 160 marks. Each question has 4 sub-questions. There are 16 sub-questions in total. The aim is to assess your ability to express knowledge concisely. Each sub-question is worth 10 marks.-8  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 28 August 2013 Paper 3 (3 hours) Instructions 1 Answer each of the following FOUR (4) questions in separate books. These are all short notes type questions. Answers to each sub-question should be brief and to the point. You may answer the questions in Afrikaans. All questions are to be answered. 2 3 4 5 .