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Chapter 68. Sickle Cell Disease

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Which of the following factors can contribute to increased risk for vasoocclusion in sickle cell disease patients?

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A. Decreased red blood cell (RBC) deformability

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B. High RBC fetal hemoglobin (HbF) concentration

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C. Low RBC adult hemoglobin (HbA) concentration

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D. Rapid RBC transit through microcirculation

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E. Decreased RBC viscosity

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MM is a 9-year-old boy with sickle cell disease (SCD) who has just had a stroke. He is starting to regain some motor function and is progressing well. What is the most effective measure that can be taken to prevent further strokes in MM?

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A. Hydroxyurea taken daily for life

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B. IV antibiotics for 10 days to prevent meningitis

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C. Anticoagulant medications

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D. Chronic transfusion RBC therapy for life

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E. Iron chelation therapy

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Which of the following choices may best help to prevent infectious complications from sickle cell disease?

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A. Immunize for S. pneumoniae and take daily oral PenVK until age 6

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B. Immunize for H. flu and take oral PenVK at the first sign of a fever

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C. Immunize for Hepatitis B and give IV antibiotics at the first sign of a fever

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D. Immunize for influenza and keep the patient adequately hydrated

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E. Immunize for N.meningitidis and take daily oral amoxicillin until age 6

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A 30-year old man with sickle cell disease presents to the ER with complaints of lethargy and tiredness increasing over the past 2 weeks. His oxygen saturation level is 86% (0.86) on room air. Hemoglobin is 5.4 g/dL (54 g/L; 3.35 mmol/L). WBC is 5 × 103/mm3 (5 × 109/L), platelets 100 × 103/mm3 (100 × 109/L). Oral temperature is 37.5°C. What one therapeutic intervention will have the best impact on his chief complaint?

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A. IV antibiotics

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B. Packed RBC transfusion

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C. IV fluids (give two times maintenance)

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D. Oxygen to keep saturations > 95% (0.95)

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E. IV morphine

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A 22-year-old ...

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