Nokea
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.
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A child with sickle cell anemia (SCA) develops severe chest and back pain, fever, a cough, and dyspnea. What should be the first action by the nurse?
- A. Administer 100% oxygen to relieve hypoxia.
- B. Notify the practitioner because chest syndrome is suspected.
- C. Infuse intravenous antibiotics as soon as cultures are obtained.
- D. Give ordered pain medication to relieve symptoms of pain episode.
Correct Answer: B
Rationale: Symptoms suggest acute chest syndrome, a medical emergency in SCA, requiring immediate practitioner notification. Oxygen may be needed but doesn?t reverse sickling, antibiotics follow evaluation, and pain management is secondary to addressing the emergency.
In a child with sickle cell anemia (SCA), adequate hydration is essential to minimize sickling and delay the vasoocclusion and hypoxia-ischemia cycle. What information should the nurse share with parents in a teaching plan?
- A. Encourage drinking.
- B. Keep accurate records of output.
- C. Check for moist mucous membranes.
- D. Monitor the concentration of the childs urine.
Correct Answer: C
Rationale: Checking for moist mucous membranes assesses hydration in SCA, where impaired kidney function prevents urine concentration. General encouragement to drink is vague, output records don?t reflect fluid needs, and urine concentration is unreliable due to kidney dysfunction.
What statement best describes b-thalassemia major (Cooley anemia)?
- A. It is an acquired hemolytic anemia.
- B. Inadequate numbers of red blood cells (RBCs) are present.
- C. Increased incidence occurs in families of Mediterranean extraction.
- D. It commonly occurs in individuals from West Africa.
Correct Answer: C
Rationale: B-thalassemia major has a higher incidence in Mediterranean populations and is inherited, not acquired. It involves overproduction of unstable RBCs, not inadequate numbers, and is distinct from sickle cell anemia, which is common in West African populations.
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.
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.