Nokea
What therapeutic intervention is most appropriate for a child with b-thalassemia major?
- A. Oxygen therapy
- B. Supplemental iron
- C. Adequate hydration
- D. Frequent blood transfusions
Correct Answer: D
Rationale: Frequent blood transfusions maintain hemoglobin above 9.5 g/dl to prevent bone marrow expansion in b-thalassemia major. Oxygen and hydration are supportive but not primary, and supplemental iron is harmful due to existing iron overload from transfusions.
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Iron overload is a side effect of chronic transfusion therapy. What treatment assists in minimizing this complication?
- A. Magnetic therapy
- B. Infusion of deferoxamine
- C. Hemoglobin electrophoresis
- D. Washing red blood cells (RBCs) to reduce iron
Correct Answer: B
Rationale: Deferoxamine infusions chelate and excrete excess iron, minimizing overload from chronic transfusions. Magnetic therapy is ineffective, hemoglobin electrophoresis is diagnostic, and washing RBCs removes other components, not iron.
In which condition are all the formed elements of the blood simultaneously depressed?
- A. Aplastic anemia
- B. Sickle cell anemia
- C. Thalassemia major
- D. Iron deficiency anemia
Correct Answer: A
Rationale: Aplastic anemia involves bone marrow failure, depressing all blood elements (RBCs, WBCs, platelets). Sickle cell anemia affects hemoglobin, thalassemia major impacts hemoglobin chain production, and iron deficiency reduces RBC size and hemoglobin, not all elements.
The clinical manifestations of sickle cell anemia (SCA) are primarily the result of which physiologic alteration?
- A. Decreased blood viscosity
- B. Deficiency in coagulation
- C. Increased red blood cell (RBC) destruction
- D. Greater affinity for oxygen
Correct Answer: C
Rationale: SCA causes increased RBC destruction due to sickled cells, leading to vasoocclusion and hemolysis. Sickled cells increase viscosity, not decrease it; coagulation is unaffected; and sickled hemoglobin has reduced oxygen affinity, transforming under low oxygen tension.
What condition occurs when the normal adult hemoglobin is partly or completely replaced by abnormal hemoglobin?
- A. Aplastic anemia
- B. Sickle cell anemia
- C. Thalassemia major
- D. Iron deficiency anemia
Correct Answer: B
Rationale: Sickle cell anemia involves replacement of normal hemoglobin with abnormal sickled hemoglobin, a hemoglobinopathy. Aplastic anemia is bone marrow failure, thalassemia major involves reduced hemoglobin chain production, and iron deficiency affects RBC size, not hemoglobin type.
The regulation of red blood cell (RBC) production is thought to be controlled by which physiologic factor?
- A. Hemoglobin
- B. Tissue hypoxia
- C. Reticulocyte count
- D. Number of RBCs
Correct Answer: B
Rationale: Tissue hypoxia triggers the kidneys to release erythropoietin, stimulating bone marrow to produce RBCs. Hemoglobin levels indirectly influence this through oxygen delivery, reticulocyte count monitors production, and RBC numbers don?t directly control production.