Sickle cell disease (NORD) Quiz: Ace Your Exams

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A 16-year-old African American boy presents to the emergency department with sudden onset of weakness, shortness of breath, and fatigue. The patient denies chest pain, and his mother explains that the patient has had symptoms of “the common cold” for the past few days. Family history includes sickle cell disease. Temperature is 37°C (98.9°F), pulse is 91/min, respirations are 19/min, and blood pressure is 126/76 mmHg. Physical examination reveals scleral icterus and pallor. Cardiopulmonary examination is within normal limits, and abdominal examination does not reveal hepatosplenomegaly. Chest x-ray is within normal limits. Laboratory tests are obtained, and the results are shown below.  
 
 Laboratory value  Result  Reference Range 
 Blood, serum, plasma   
 Sodium  136 mEq/L  136-146 mEq/L 
 Potassium  3.5 mEq/L  3.5-5 mEq/L 
 Chloride  105 mEq/L  95-105 mEq/L 
 Bicarbonate  22 mEq/L  22-28 mEq/L 
 Blood urea nitrogen (BUN)  15 mg/dL  7-18 mg/dL 
 Creatinine  1.0 mg/dL  0.6-1.2 mg/dL 
 Glucose  119 mg/dL  70-110 mg/dL 
 Hematologic   
 Hemoglobin  3.5 g/dL  13.5-17.5 g/dL 
 Hematocrit  33%  41-53% 
 Reticulocytes  0.1%  0.5-1.5% 
 Leukocyte count  12,000/mm3  4,500-11,000/mm3 
 Platelet count  200,000/mm3  150,000-400,000/mm3 
   
Which of the following is the most likely diagnosis?  

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