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

7 September 2011 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. ______________________________________________________ Question 1

Mahlori is a 10-year-old girl who is a well-controlled asthmatic. She presents to casualty with a two-hour history of facial swelling and cough. Shortly after arrival she is unable to open her eyes because of the swelling, has developed an urticarial rash and has bronchospasm. She is diagnosed as having acute anaphylaxis a) b) Describe the immunology and pathophysiology of anaphylaxis. (5)

Name two other systems that may be involved in acute anaphylaxis, and list the presenting features in each system. (5) List 3 common precipitating factors for anaphylaxis in children. What is the emergency management of anaphylaxis? (3) (5)

c) d) e)

Describethemechanism of action and two common side effects of THREE drugs you may choose to manage anaphylaxis. (8) Discuss how you would prevent recurrence of this episode (4) [30]

f)

Question 2 Tshegofatso, an 11-month-old girl, is admitted with a 10 day history of diarrhoea. Her mother had been providing an appropriate salt-sugar solution. She is no longer breastfeeding. She is inattentive of her surroundings. She is hypotonic and weak; her

mother reports that she became weak yesterday. Anthropometric assessment yields a weight-for-age z-score of -3.1 and a length for age z-score of -2.1 a) Interpret the two z-scores. An initial urea and electrolyte result shows: Sodium134mmol/l, Potassium 1.8mmol/l, Chloride 101mmol/l, Urea 6.5mmol/l What is the pathophysiology of Tshegofatsos hypokalaemia? (4)

b) c)

(2)

How does hypokalaemia cause weakness? (2) An intravenous infusion of strength Darrows Dextrose ( DD) solution is started. A decision is made to increase the potassium concentration of the infusion to40mmol/l. What are the constituents of DD solution? (2)

d) e)

What volume of 15% potassium chloride solution must be added to each 500ml of DD in order to achieve this concentration of potassium? Indicate how this is calculated. (Note: 75 mg KCl = 1 mmolKCl). (2) The next day Tshegofatso is more alert, her hydration status is clinically normal and her muscle strength has improved, but her diarrhoea is no better. During the course ofthe day she develops carpopedal spasms and extensor posturing. A quick review reveals a widely patent but flat anterior fontanelle. A repeat urea and electrolytes test shows: Sodium 137mmol/l, Potassium 2.7mmol/l, Chloride 103mmol/l, Urea 4.3mmol/l,Total Calcium 1.55mmol/l, Phosphate 0.8mmol/l, Magnesium 0.6mmol/l What is the cause of her carpopedal spasm and posturing? Discuss the regulation of serum calcium. (1) (5)

f) g) h)

What might be causing Tshegofatsos calcium abnormality? (2) An infusion of calcium gluconate is administered and Tshegofatso responds well to this. Ten days later she still has diarrhoea and her diet is changed from a cows milk formula and solids to soya milk and dairy free solids. Offer a reason why her diet has been modified. How would you confirm the suspicion that this decision is based upon? (1) (1)

i) j) k)

Discuss the pathogenesis and clinical relevance of the possible condition necessitating the dietary change. (4) A zinc supplement is added to her treatment. List two clinical benefits of zinc in a child with diarrhoea. List two physiological actions of zinc that allow these beneficial effects. (2) (2) [30]

l) m)

Question 3 Pieter, an 18-month-old boy, presents to the paediatric admission ward with a 2 dayhistory of diarrhoea with blood and mucous in the stool. He is pyrexial and irritable. E. coli is suspected as the most likely cause of his diarrhoea. The treating clinician decides not to administer antibiotics.

a) b)

List three bacteria, other than E. coli, that could be responsible.

(3)

Discuss the advantages and disadvantages of administering antibiotics to children with bloody diarrhoea. (3) How does E.coli cause diarrhoea? Briefly describe five different mechanisms. (5)

c)

On day 2 of admission, Pieter looks pale and his mother says he has had a dry nappy for more than 12 hours despite good intake of fluids. d) What complication do you suspect Pieter has developed? Pieter develops petechiae, and bruises easily during venepuncture. A full blood count and clotting profile is performed. e) Predict the results (normal/increased/decreased) for each of the following i) Haemoglobin. ii) Reticulocytes. iii) INR. What is the pathogenesis of each of these changes? His biochemistry shows a potassium of 7.0mmol/l. f) Outline the emergency management of hyperkalaemia. His condition worsens and it is decided to commence dialysis. g) h) List five indications for dialysis. Describe the physiological principles for how peritoneal dialysis works. Pieter has a generalized tonic clonic seizure. j) Indicate three possible causes for the seizure. (3) [30] (5) (3) (4) (1)

(3)

Question 4 Baby J is a 3-day-old infant who is brought to the local clinic because his mother is worried about the yellow discoloration of his eyes and skin. He was born at 33 weeks gestation weighing 1800 grams. His mother tested Rhesus-negative. He has been exclusivelybreastfed since birth. A serum bilirubin level is measured and he is placed under phototherapy. a) b) c) Draw a diagram of bilirubin metabolism in a neonate. Briefly explain entero-hepatic circulation. (4) (2)

List three reasons why a preterm infant is more likely to develop neonatal jaundice than a term infant. (3) Briefly explain the mechanism of iso-immune haemolysis. (4)

d) e)

List two important laboratory investigations you would perform to establish the cause of the jaundice, and the value of each test. (2)

f) g) h)

Explain how breastfeeding predisposes to the development of neonatal jaundice. (3) Explain the mechanisms of action of phototherapy and exchange transfusion. (4)

List TWO drugs, together with their mechanism of action, which can be used in the treatment of neonatal jaundice. (4) Discuss thepathophysiology ofbilirubin-induced neurological dysfunction (BIND). (4) [30]

i)

Question 5 Karabello, a 19-month-old girl presents with a one week history of fever, irritability, decreased appetite and vomiting. On examination her Glasgow Coma Scale (GCS) score is 13 and she has meningism. Her weight-for-height Z score is -3.4. A lumbar puncture is performed. a) Name the different anatomical layers traversed by the lumbar puncture needle until cerebrospinal fluid is obtained. (2) What is the most common explanation for a blood-stained CSF tap? What are the three components of a Glasgow Coma Scale assessment? How would you interpret a GCS of 13? The CSF result is suggestive of tuberculous meningitis. (1) (1) ()

b) c) d)

e)

Regarding patients with tuberculous meningitis, describe the changes typically found in CSF biochemistry and microscopy. (2) Briefly explain the pathophysiology of the CSF abnormalities. (4)

f) g)

Briefly discuss the immune response to primary Mycobacterium tuberculosis infection. (5) Karabellos tuberculin skin test (TST) is non-reactive. Her HIV Elisa test is also negative. Discuss the immunological mechanism of the tuberculin skin test. Offer one reason why KarabellosTST may be falsely negative. Karabello is started on anti-tuberculous treatment. (3) (1)

h) i)

j)

Regarding the pharmacology of isoniazid, discuss i) Its mechanism of action. ii) Two common side effects and their management. Three days after admission, Karabellos level of consciousness worsens. A CT scan demonstrates hydrocephalus.

(5)

k)

Explain the difference hydrocephalus.

between

communicating

and

non-communicating (1) (2)

l)

What is the pathogenesis of hydrocephalus in tuberculous meningitis?

In an effort to manage the hydrocephalus, furosemide and acetazolamide are added to Karabellos treatment regimen. m) Why would each of these be used? i) Furosemide. ii) Acetazolamide.

(2) [30]

Question 6 A 25-year-old primigravida gives birth to a male term neonate who is diagnosed with Trisomy 21. The neonate has no problems at birth. At 6 months of age he is noted to have peripheral oedema, hypotonia, keratosis and constipation. a) Explain the genetic mechanisms underlying the various chromosomal abnormalities that are collectively referred to as Trisomy 21. Which is most likely in this child? (4) What is the recurrence risk of each of these chromosomal abnormalities? Discuss the laboratory diagnosis of Trisomy 21. (2) (2)

b) c) d)

List FOUR screening tests relevant to the antenatal detection of Trisomy 21 in this mothers subsequent pregnancies. (4) Chromosome 21 is considered to be an acrocentric autosome. What does this mean? (1) Briefly discuss the possible haematological manifestations of Trisomy 21. What endocrine complication has occurred at 6 months of age? What is the pathophysiology of this endocrine condition? How would you monitor and treat the endocrine condition? (3) (1) (2) (3)

e)

f) g) h) i) j)

This child requires a surgical procedure and the anaesthetic is to be administered by a junior doctor. What advice would you offer this doctor regarding possible intubation risk and difficulty? (3) At 1-year of age this child develops a severe community-acquired pneumonia but is refused ICU admission.

k)

Do you agree with this decision? Justify your reasoning.

(5) [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

8 September 2011 Paper 3 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 should not spend more than 11 minutes per sub-question. These are all short notes 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. ______________________________________________________ Question 1 a) b)

__________

Write a brief description of the five stages in the embryology of the lung. (10) (i) Label the items numbered 1 8 in the spirogram below. (4) (ii) Briefly explain the physiological relevance of measurements 3, 5 and 8.(6)

c)

(i) Draw the oxygen-dissociation curve for haemoglobin. (ii) Indicate how any two physiological parameters alter this curve. (iii) Explain the clinical relevance of these alterations.

(6) (2) (2)

d)

A two year old boy is bitten by a rabid dog. Discuss measures to prevent rabies developing in this child, including the scientific basis for offering each intervention. (10) [40]

Question 2 a) b) Discuss the pathophysiology of septic shock. (10)

In a child with diabetic ketoacidosis, discuss the pathogenesis of each of the following (i) Alteration in level of hydration. (ii) Acid-base disturbance. (iii) Changes in potassium homeostasis. (10) At 4 years of age a child is diagnosed with an inoperable ventricular septal defect. His heart was assessed to be clinically normal at birth. At age 12years he is cyanosed. Discuss the cardiovascular physiological dynamics (systemic and pulmonary vascular resistance, blood flow and pressure changes) explaining the findings at all three time-points. (10) List the different levels of evidence that may be used in formulating a clinical guideline, together with a short description of each level. (10) [40]

c)

d)

Question 3 Write short notes on: a) The pharmacokinetics (absorption, distribution, metabolism and excretion) of aminoglycosides in paediatrics. (10) Glomerular filtration rate: include definition, estimation/calculation and clinical relevance. (10) The diagnosis and prevention of pertussis. i) ii) (10)

b)

c) d)

Write short notes on IgM or IgA, including a labeled diagram of the molecule. Name the other (apart from IgA and IgM) classes of immunoglobulins. (10) [40]

Question 4 Write short notes on: a) ONE non-steroidal anti-inflammatory drug (NSAID) under the following headings i) Mechanism of Action. ii) Indications for use in paediatrics. iii) Side effects. iv) Drug interactions. (10) HIV-associated Immune Reconstitution Inflammatory Syndrome (IRIS). (10) Normal social and emotional developmental milestones at 3, 7 and 12 months of age. (10)

b) c)

d)

i) ii)

Draw a labelled diagram of the neuromuscular junction. Indicate on the diagram where the abnormality related to each of these occurs. i. Myasthenia gravis. ii. Organophosphate poisoning. iii. Tetanus. iv. Botulism. (10) [40]