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Chapter 68. Sickle Cell Disease

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

A. Decreased red blood cell (RBC) deformability

B. High RBC fetal hemoglobin (HbF) concentration

C. Low RBC adult hemoglobin (HbA) concentration

D. Rapid RBC transit through microcirculation

E. Decreased RBC viscosity

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?

A. Hydroxyurea taken daily for life

B. IV antibiotics for 10 days to prevent meningitis

C. Anticoagulant medications

D. Chronic transfusion RBC therapy for life

E. Iron chelation therapy

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

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

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

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

D. Immunize for influenza and keep the patient adequately hydrated

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

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?

A. IV antibiotics

B. Packed RBC transfusion

C. IV fluids (give two times maintenance)

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

E. IV morphine

A 22-year-old ...

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