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 March 2012 Paper 2 Instructions 1 2 3 Answer each of the following FIVE(5) questions in separate books. All questions are to be answered. Each question is worth 30 marks you should not spend more than 30 minutes per question. The aim is to assess your ability to express knowledge concisely and precisely. __________ (3 hours)

4 You may answer the questions in Afrikaans, if you wish. ______________________________________________________ Question 1

a) b)

An eighteen-month-old male infant presents to POPD with delayed milestones. On examination he has frontal bossing, thickened wrists and patent anterior fontanel. A diagnosis of rickets as made. What is the role of Vitamin D in calcium and phosphate homeostasis? (5) List 5 causes of delayed closure of the anterior fontanel. (5)

An x-ray was done of the wrists and knees and confirmed the clinical diagnosis. Laboratory findings showed the following Calcium: 2.19mmol/L Phosphate: 0.7mmol/L Magnesium: 0.72mmol/L Intact Parathyroid Hormone: 123pg/mL c) d) e) f) (N = 2.25 2.75mmol/L) (N = 1.25 2.10mmol/L) (N = 0.6 0.95mmol/L) (N = 1 43pg/mL) (5) (5) (5) (5) [30]

What are the radiological features of rickets? Tabulate the biochemical abnormalities in the different types of rickets. Discuss the management of the above patient. Discuss the role of vitamin D therapy in chronic kidney disease.

PTO/Page 2 Question 2

-2Question 2 Twelve grade 4 children from the same school present to the casualty department with a history of nausea, vomiting and severe abdominal cramps for the previous two hours. They have been on a school camp that started three days earlier. You suspect food poisoning. a) Complete the following table by providing the missing information in the appropriate boxes labeled A-H that relate to clinical syndromes associated with foodborne diseases and may aid you in coming to a diagnosis for the 12 children. Clinical Syndrome Nausea and vomiting Neuromuscular weakness, gastrointestinal tract manifestations Haemolytic-uremic syndrome E Guillain Barr syndrome Fever, malaise anorexia, jaundice Nausea and vomiting Incubation Period 1 6 hours 12-48 hours Causative Agent A B Commonly associated vehicles Fried rice, meats Home canned vegetables, fruits and fish, salted fish, meats, bottled garlic, cheese sauce, honey (infants) D

Varied 6-24h Varied 15-50 days H

Mushroom toxins (late Mushrooms (especially onset) Amanita species) Poultry, raw milk , water F G Staphylococcus aureus Shellfish, raw produce (e.g. lettuce) Ham poultry, cream filled pastries, potato and egg salads (8)

b)

The casualty officer has prescribed the following treatment: Loperamide (Immodium), Cyclizine (Valoid), Trimethoprim-sulfamethoxazole (Bactrim) Discuss each of these three drugs under the headings i) Mechanism of action. (3) ii) Adverse effects. (6) iii) Appropriateness of their prescription in the scenario as described above. (6) The children apparently played in a water stream close to the camp. There was a cholera outbreak in the area during the previous year.

c)

Briefly discuss cholera under the headings i) Treatment. ii) Prevention .

(2) (3)

PTO/Page 3 Question 2d) -3-

A diagnosis of toxin mediated staphylococcal food poisoning is made. d) Name 2 other toxin mediated staphylococcal diseases. (2) [30]

Question 3 Naledi is referred at the age of 6-years because of her height. She has always been smaller than her siblings and friends and her mother feels her growth has been slower than before. a) There are four main influences which may have an impact on her poor growth. Briefly discuss the possible roles of i) Genetic factors. (4) ii) Nutritional factors. (4) iii) Hormonal factors. (4) iv) Environmental factors. (6) On examination she is slightly overweight with a growth velocity of 3cm / year. Her maximal growth hormone response is very poor. b) c) Name 5 causes of growth hormone deficiency. (5)

Briefly discuss the mechanism of action and common side effects of growth hormone replacement therapy. (7) [30]

Question 4 An 11-year-old Black child is referred to his paediatrician for a problem of headache, abdominal pain and easy fatigability. On examination he is found to have diminished arterial pulses in both his lower limbs, systemic hypertension (average blood pressure: 180/120mmHg) and proteinuria 2+ on dipsticks analysis. He also has clinical evidence of cardiomegaly with a thrusting apex beat. He has no signs of heart failure and his fundal examination is normal. List the possible causes and investigations required to diagnose this childs problem. (10) Discuss briefly the pathogenesis of primary hypertension in children. (10)

a)

b)

The child has a seizure in the ward and an MRI scan shows evidence of cerebral oedema but no evidence of intracranial haemorrhage. The seizure was controlled with intravenous diazepam. Post seizure there is no evidence of neurological deficit. c) Discuss briefly the pathogenesis of hypertensive encephalopathy. (10) [30]

PTO/Page 4 Question 5

-4Question 5 A 4-month-old infant is brought by her mother to the paediatrician because the mother feels she is not gaining weight adequately, has a fever and a pellagra-like rash involving her perineum and elbows. The child is breastfeeding and the mother has not yet introduced solids. Mom is particular concerned about the immune status of the child since she was diagnosed as being HIV positive a month before. The infant is subsequently found to be HIV positive. a) Discuss the pathogenesis of poor growth in HIV-infected children. (10) b) c) Discuss the effects of zinc on the immune system. Discuss the advantages of exclusive breastfeeding in HIV positive infants. (5) (5)

On examination the child is found to be pyrexial with failure to thrive and has generalised lymphadenopathy with hepatosplenomegaly. Chest x-ray shows bilateral pneumonia, confirmed to be due to cytomegalovirus. d) Discuss briefly the management of CMV pneumonia in infants. (10) [30]

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

29 March 2012 Paper 3 Instructions 1 2 3 4 Answer each of the following FIVE(5) 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. (3 hours)

5 You may answer the questions in Afrikaans, if you wish. ______________________________________________________ 1

__________

Julie is a five-year-old girl has a weight of 40 kg and her length is 119 cm. a) Define body mass index and explain how it is used to classify overweight obese children. b) Describe the methods used in body fat measurement. c) Discuss the hormones involved in food intake regulation. d) Discuss the endocrine functions of adipose tissue. e) Discuss the medical complications of obesity.

and (5) (10) (10) (5) (10) [40]

a) Briefly describe the mechanisms that lead to abnormal calcium and phosphorus homeostasis in Chronic Kidney Disease (CKD). (10) b) Regarding Chronic Kidney Disease (CKD) and hyperkalaemia i) Briefly describe the pathophysiologic mechanism causing hyperkalaemia in CKD. (3) ii) What are the electrocardiographic features of hyperkalaemia? (2) iii) List and describe the potential adverse effects of the various pharmacological agents that are used to treat hyperkalaemia. (5) c) Regarding conjunctivitis i) Describe the local natural defence mechanisms that protect the eye against conjunctivitis. (5) ii) Describe the findings of a Gram stain performed on a sample of purulent conjunctival discharge obtained from a neonate with Neisseria infection. (1)

PTO/Page 2 Question 2iii) -2iii) List two appropriate prophylactic regimens that are used to prevent neonatal conjunctivitis. (2) iv) How is neonatal N gonorrhoea conjunctivitis treated? (2) v) Write a short note on hepcidin in relation to iron metabolism. (5) vi) Write a short note on fibroblast growth factor-23 (FGF-23) in relation to phosphate metabolism. (5) [40] 3 A full term neonate presents with a blood glucose of 2 mmol/L at 2 hours of age. a) Discuss the key hormones involved and the post natal changes expected to occur to counteract the low blood glucose. (10) b) Discuss the risk factors for impaired metabolic adaptation in neonates for the following metabolic processes i) Glycogenolysis. (4) ii) Gluconeogenesis. (4) iii) Lipolysis. (4) iv) Ketogenesis. (4) c) Explain why a urinary ketone level helps to distinguish different causes of hypoglycemia (4) The baby needs 12 mg/kg/minute of glucose to maintain a normal blood glucose level and he has an elevated serum insulin level. d) Name 4 medications which may be useful in the scenario and give their mechanism of action. (10) [40] Birth trauma is a common following vacuum extraction in term neonates. a) Discuss the complications of birth trauma following vacuum extraction in neonates. (10) b) Discuss the management of any two of the above complications (20) c) Discuss the value of APGAR scores. (10) [40] 5 A 1-year-old child presents with features of lower motor neuron lesion since birth. a) Describe the lower motor unit? (10) b) Give two examples of diseases affecting each component of the lower motor unit. (5) c) Discuss the pathogenesis of Myaesthenia gravis. (10) d) Discuss the role of pharmacological agents in the management of lower motor neuron lesions in children. (15) [40]