1. OCSE Peadiatrics

2. OCSE 1

3. i ii iii iv

4. Questions 1.Interpret the pedigree ( 3marks ) 2.State the type of inheritance showed in the pedigree. ( 2 marks ) 3.Give 2 example of disorders associated with this inheritance. ( 2 Marks ) 4.Discuss the risk of the offspring of the patient if she marries a normal person. ( 3marks )

5. Answers 1.Only males are affected, females are either carriers or unaffected, no male to male transmission. 2.X-linked ressesive inheritance 3.Hemophilia, Duchene Muscular Dystrophy, Color Blindness, Glucose 6 Phosphate Dehydrogenase Deficiency 4.For a carrier female of the disorder having children with a normal male, each son has 50% chance of being affected and each daughter has 50% chance of being a carrier.

6. OCSE 2

7. The chest radiograph below belongs to a 2 year old infant presented with dyspnea on exertion and relieved by squatting

8. Questions 1.List the findings of the chest radiograph. (2 marks) 2.State the clinical features you expect from this patient (3 marks) 3.What is the provisional diagnosis? (2 mark) 4.State the emergency management for this patient ( 3marks )

9. Answers 1.List the findings of the chest radiograph. (2 marks) Heart size normal Cardiac apex displaced upward Decreased pulmonary vascularity Pulmonary artery segment concave

10. Answers 2.What clinical features you expect from this patient? (3 marks) Failure to thrive Cyanosis of the lips and nail bed Fingers and toes clubbing A harsh ESM is heard over the pulmonic area and the left sternal border

11. Answers 3.What is the provisional diagnosis? (1 mark) Tetralogy of Fallot

12. Answers Emergency Medical treatment Hypercyanotic spells- One or more of the following in sequence – place in knee-chest position – 100% O2 (mask) – IV Propranolol 0.1 -0.2 mg/kg & then prophylactic dose 0.5-1 mg / kg /dose 2-3 times a day orally – S.C morphine 0.1 mg/kg – IV Sodium bicarbonate – Vasopressors- IV phenylephadrine/ methoxamine Correct iron deficiency anaemia Treat polycythemia – adequate hydration Venesection Partial exchange transfusion

13. OSCE Farah is a 12 year old girl who was brought to the clinic with what her parents describe as frequent “stare”. These occur many times in a day. She is otherwise well. Watch this video of Farah and answer the following questions

14. Questions 1.What is your provisional diagnosis? ( 2 Mark ) 2. Mention the features suggest the diagnosis in this video (3marks) 3. Mention 2 investigations you would like to do ( 2 Marks ) 4. How would you treat the condition mentioned above? ( 3marks )

15. 1. Absence seizure (Petit mal seizure) 2. Signs of absence seizures in Farah include: – Vacant stare – Eyelid flutters – Up-rolling of eyeball – Absence seizures last only a few seconds. – Full recovery is almost instantaneous.

16. 3. Mention 2 investigations you would like to do Metabolic screen Toxicology screen EEG can be diagnostic MRI

17. Treatment Antiepileptic drugs (AEDs) Ethosuximide (Zarontin) and valproic acid (Depakene, Depacon)

18. Physical activity should not be restricted more than necessary Activities in which a seizure might pose a threat, such as swimming or rock climbing, may be allowed with appropriate supervision. A child with epilepsy should not be unnecessarily handicapped Compliance to medication Patients with uncontrolled absence seizures should not be allowed to drive.

19. Thank you!