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 9 April 2013 Paper 2 Instructions 1 2 3 Answer each of the following SIX (6) 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. ______________________________________________________________________________

-2Question 1 Miriam, a 12-year-old girl, has an argument with her father. A few hours later she is brought to the emergency department by her mother, who has noticed progressive lethargy, drowsiness and disorientation. Miriam complains of abdominal pain and ringing in her ears. On examination she is pale, but not jaundiced. Her temperature is 380C, pulse rate is 125bpm, blood pressure is 100/50mmHg and blood sugar is 5mmol/L. Her level of consciousness is reduced. She opens her eyes to voice, is able to localise painful stimuli, but talks incoherently. Her pupils are dilated, equal and reactive. a) What is her Glasgow Coma Scale (GCS), indicating the individual scores for each component? (3)

Her arterial blood gas while breathing ambient (room) air reveals: pH = 7.48, Pa0 2 = 9.8kPa (74mmHg), PaCO 2 = 2.6kPa (20mmHg), HCO 3 - = 20mmol/L, Base Excess = -2.9 b) Assess her acid base status. (2)

Four hours later she is more confused. Her blood gas is repeated. pH = 7.22, PaCO 2 = 3.7kPa (28mmHg), Pa0 2 = 10kPA (75mmHg), HCO 3 - = 12mmol/L, Base Excess = -13 c) Assess her acid base status now. (3)

Results of some earlier investigations are now available Full Blood Count: Hb = 10g/dL, WCC = 8 x109/L, Platelets = 222 x 109/L Liver Function Tests: total protein = 74g/L(range 60-80g/L); albumin = 40g/L(range 29-42g/L), total bilirubin = 5mol/L(range 0-21mol/L), aspartate aminotransaminase (AST) = 40U/L; (range 0-37U/L); alanine aminotransferase (ALT) = 20U/L (5-20U/L); gamma glutamyltransferase (GGT) =20U/L(3-22U/L); alkaline phosphatase = 125U/L (51-332U/L INR = 1.25 Sodium = 143mmol/L, Potassium = 2.8mmol/L, Chloride = 101mmol/L, CO 2 = 13mmol/L, Urea = 9.2mmol/L, Creatinine = 128mol/L d) e) f) Calculate and interpret her anion gap. List 3 causes of an abnormal anion gap. Calculate and interpret her serum osmolality. (4) (3) (3)

Miriams father arrives with an empty container of his analgesic tablets. It was full earlier in the day. g) h) i) What is the likely toxin? (1) Describe its pathophysiological mechanisms and indicate how this explains the relevant serum chemistry results. (4) Briefly discuss the principles of Miriams further management. (7) [30]

PTO/Page 3 Question 2

-3Question 2 You are called to Labour Ward to see a newborn with respiratory distress. She has a birth weight of 3.05kg. Her Apgars are 5 and 7 at 5 and 10 minutes respectively. She is cyanosed despite bag-mask ventilation with a pulse oximetry saturation of 69%. You notice a scaphoid abdomen and are concerned about congenital diaphragmatic hernia. a) b) c) d) e) f) g) What clinical sign(s) might you elicit on chest examination? (2) Briefly discuss (with the aid of a diagram if preferred), the normal embryological development of the diaphragm. (4) List 2 types of congenital diaphragmatic hernia. (2) Identify the embryological origin of each. (2) What is the respiratory function pathophysiology of a congenital diaphragmatic hernia? (3) List TWO abnormalities you would you expect to find on her chest x-ray. (2) What TWO pathological features would you expect to find, if you obtained a pathological sample of the affected lung? (2)

You decide to intubate and ventilate the infant but the saturations improve to only 79% despite maximal ventilation. h) Provide TWO possible explanations for her low oxygen (pulse oximetry) saturation. (2)

Pulse oximetry saturation measured in the right hand is noted to be 85%, while that of the left hand is 69% i) Explain the differential saturations. (2)

A blood gas is done pH = 7.00 PaCO 2 = 77mmHg (10,2kPa), P a O 2 = 43mHg(5,7kPa), Base Excess = -2mmo/L Standard bicarbonate = 22mmol/L Her blood pressure = 40/20mmHg j) Indicate strategies you would use to correct the hypotension, acidosis, hypercarbia and hypoxia. (8) After 6 hours her condition suddenly deteriorates and fresh blood is suctioned from her endotracheal tube. k) What do you suspect has now happened? (1) [30]

PTO/Page 4 Question 3

-4Question 3 Siviwe, a 6-year-old boy is brought to the hospital by his mother. He has been unwell for 2 weeks. When he became ill, Siviwe was seen at the local clinic and found to have tonsillitis. Oral Penicillin was prescribed. a) b) Name the class of antibiotics to which penicillin belongs, name 2 other antibiotic agents in this class. (2) What is the mechanism of action of these antibiotics? (2)

Siviwe is not getting better. His mother says that over the past few days, his body (especially his face) has become swollen, and his urine is now brown. On examination, he has generalised oedema and dyspnoea. His heart rate is 120bpm, his blood pressure is 160/110mmHg and his heart sounds are normal. A diagnosis of acute post-infectious glomerulonephritis is made. c) d) What is the mechanism of the oedema? (2) Based on streptococcal antigenic properties, what investigations could you do to confirm preceding streptococcal infection? (2)

You request serum complement levels. d) i) ii) What is the rationale for this? (3) Indicate the immediate effect you expect (increased, decreased or normal) on the levels of total complement, C3 and C4. (3)

While you are examining Siviwe, he has a generalised tonic-clonic seizure. e) What is the most likely cause of his seizure? f) Briefly explain the pathophysiology of his seizure.

(1) (4)

On further examination you find clinical features of fluid overload with anuria. Serum chemistry results include the following Sodium = 120mmol/L (range 135-145mmol/L); Potassium = 6.5mmol/L (range 3.5-5mmol/L); Chloride = 100mmol/L (range 98-106mmol/L) Corrected Calcium = 1,8mmol/L (range 2.2-2,7mmol/L); Phosphate = 3.4mmol/L (range 0.871.45mmol/L) Urea = 26mmol/L (range = 1.8-6.4mmol/L); Creatinine = 203mol/L (range = 27-62mol/L) g) Briefly explain each of the following abnormal results i) Sodium. ii) Potassium. iii) Corrected Calcium. iv) Phosphate. v) Urea. vi) Creatinine. (8)

Intravenous furosemide is prescribed. h) Outline the mechanism of action of furosemide. (3) [30] PTO/Page 5 Question 4

-5Question 4 A 13-year-old boy presents with weakness in his left arm, of a few days duration. When questioned, he admits to recent tiredness. On examination, he has a left hemiplegia. Investigations are requested, and he is found to have a white cell count of 300 000 (300 x 109 cells/L). You suspect he has acute leukaemia. a) What is the likely explanation for his hemiplegia? (2)

Treatment commences. He becomes hypertensive and oliguric. You suspect tumour lysis syndrome. b) List 3 laboratory abnormalities found with tumour lysis syndrome, and explain the pathophysiology of each (6)

The patients haemoglobin is 5g/dL, and he is tachycardic, with cardiomegaly and a gallop rhythm. c) Describe the precautions you would take when transfusing packed red cells in this patient. Explain the rationale for each of these precautions. (6)

The patient receives a packed red cell transfusion and then develops a fever. d) List 2 possible explanations for his fever. (2)

The patient requires dialysis. His HIV test is positive, and the nephrology team decides not to dialyse him. e) Do you agree with this decision? Using ethical principles, justify your response. (5)

His chemotherapy regimen includes high dose prednisone. f) List 3 short and 3 long term complications of prednisone use in children. (6)

After initial chemotherapy, his white cell count falls to 1 x 109/L, with a neutrophil percentage of 10%. He spikes a temperature to 39.5C, and on examination, no source of sepsis is found. g) What is the difference between febrile neutropaenia and neutropaenic sepsis? (2)

A few weeks later, he is again febrile and neutropaenic and develops respiratory distress with low oxygen saturation. h) Name two organisms that may be implicated in the development of this complication.(1)

[30]

PTO/Page 6 Question 5

-6Question 5 Amila is a 3-year-old girl who presents to the outpatient department with a concern of pain in her vaginal area and a 2 day history of a brownish vaginal discharge. She currently attends crche during the day and all her immunisations are up to date. On general examination she is found to be Tanner Stage 1. a) Describe Tanner stage 1 in females and males. (5)

On genital examination she has inflamed labia minora with a thick white vaginal discharge. Vulvovaginitis is diagnosed. b) c) d) Briefly describe normal vaginal mucosal immunity. Name three organisms implicated in the aetiology of vulvovaganitis. Name a pharmacological agent active against each of these organisms. (6) (3) (3)

You are concerned about possible sexual abuse and perform a genital examination. e) Identify each of the numbered structures. (7)

1 2 3 4 6 7 5

You decide to provide Post Exposure Prophylaxis for HIV. f) List 2 pharmacological agents you would choose and 2 side effects for each. (6) [30]

PTO/Page 7 Question 6

-7Question 6 A 5-year-old boy with a dilated cardiomyopathy is in chronic cardiac failure and complains of persistent abdominal pain. He has intermittent vomiting and the abdomen while soft, is somewhat distended. The liver is enlarged. a) Describe (use diagrams if you find that helpful) the anatomy of the vascular supply of the stomach and intestines (including both the arterial supply and the venous drainage). (6)

His abdomen becomes more tense, and there is concern about abdominal compartment syndrome. b) What is the abdominal compartment syndrome. Please discuss in the context of abdominal organ function and intra-abdominal pressure. (6)

An x-ray done soon after admission shows a large right sided pleural effusion. A pleural tap is done in order to drain some fluid, and for diagnostic purposes. c) Describe the anatomy of the chest wall and diaphragm with reference to doing a safe pleural tap. (4)

When tapped, it is noted that the fluid is milky. The fluid contains high levels of triglycerides and numerous lymphocytes. d) e) f) g) What is the nature of the effusion? Explain the pathogenesis of the effusion in this patient. Describe how lipids are absorbed from the gut and then transported to tissues. (2) (3) (5)

Describe what therapy could be instituted in order to resolve the effusion and indicate how the therapy relates to the pathogenesis of the effusion. (4) [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 10 April 2013 Paper 3 (3 hours)

Instructions 1 2 3 4 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 not spend more than 11 minutes per sub-question. should

These are all short notes type questions. The aim is to assess your ability to express knowledge concisely. You may answer the questions in Afrikaans, if you wish.

_____________________________________________________________________________________

-21 Write short notes on a) The innate and adaptive immune responses to tuberculosis. b) The definition and causes of eosinophilia in children. c) Laboratory investigation of haemolytic anaemia in a neonate. d) The pathogenesis and clinical stages of pertussis.

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

Write short notes on a) Phosphodiesterase 5 inhibitors (e.g. sildenafil), including mechanism of action, indications for use in paediatrics and side effects. (10) b) Nasal CPAP: include mechanism of action and adverse effects. (10) c) Thermoregulation in the neonate. (10) d) Umbilical venous cannulation, including relevant anatomy and complications. (10) [40] Write short notes on a) Pathogenesis, clinical consequences and prevention of refeeding syndrome. b) Carnitine deficiency. c) Monitoring the child with diabetes. d) Vitamin D synthesis and physiological actions.

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

a) b) c) d)

Define shock. Classify shock and give an example of each type. (10) Describe the cardiac cycle with the aid of a diagram . (10) You are electively intubating a child with pneumonia. Discuss the actions and sideeffects of THREE drugs you would use to assist your intubation. (10) Briefly describe the diagnosis of i) Central diabetes insipidus. ii) Syndrome of inappropriate anti-diuretic hormone secretion (SIADH). (10) [40]