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Question 1
A 12-month-old infant of African descent undergoes screening for hemoglobinopathy. High-performance liquid chromatography (HPLC) reveals HbA 0%, HbS 88%, HbF 9%, and HbA2 3.0%. Both parents are confirmed carriers of the sickle cell trait (HbAS). Which diagnosis is most consistent with these laboratory findings?
Correct Answer:
Homozygous Sickle Cell Anaemia (HbSS)
Question 2
A 4-year-old child with sickle cell anaemia (HbSS) is initiated on hydroxyurea disease-modifying therapy. Which physiological mechanism accounts for the primary therapeutic benefit of hydroxyurea in reducing vaso-occlusive pain crises?
Correct Answer:
Induction of gamma-globin gene expression and increased fetal hemoglobin (HbF)
Question 3
A 6-year-old boy with HbSS presents to the emergency department with fever (38.9°C), cough, chest pain, tachypnoea, and a new segmental infiltrate on chest radiography. Pulse oximetry shows oxygen saturation of 91% on room air. What is the immediate first-line medical management package?
Correct Answer:
Supplemental oxygen, empiric IV ceftriaxone plus oral azithromycin, analgesia, and simple blood transfusion
Question 4
Annual Transcranial Doppler (TCD) screening in a 5-year-old girl with sickle cell anaemia demonstrates a time-averaged maximum mean velocity (TAMMV) of 215 cm/s in the right middle cerebral artery. According to international guidelines (STOP trial criteria), what is the most appropriate long-term management strategy?
Correct Answer:
Initiation of a regular chronic blood transfusion program to maintain HbS < 30%
Question 5
An 8-month-old infant presents with progressive pallor, failure to thrive, hepatosplenomegaly, and severe microcytic hypochromic anaemia (Hb 4.8 g/dL, MCV 54 fL). Hemoglobin analysis reveals HbF 94%, HbA2 4.5%, and HbA 0%. What is the underlying genetic condition?
Correct Answer:
Beta-Thalassemia Major (β0/β0)
Question 1
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Prepare with the Cert Clin Haematology(SA) Paed Practice Questions - CMSA Sub-specialty Certificate in Clinical Haematology (Paediatrics) Exam practice quiz. This question bank includes 100 questions covering year-old, presents, child, reveals, and anaemia. Use it to review important concepts, identify knowledge gaps, and build confidence for the related exam, course, or assessment.

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Cert Clin Haematology(SA) Paed Practice Questions - CMSA Sub-specialty Certificate in Clinical Haematology (Paediatrics) Exam

This practice set contains 100 questions from the matching question bank and focuses on year-old, presents, child, reveals, and anaemia. Work through each question carefully, review the provided solutions, and revisit topics that need more study before your next attempt.

This is an independent study resource intended for practice and review; it is not an official examination or an endorsement by any organization named in the title.

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