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Question: Heme

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You are seeing a 12 year old girl with PMH of sickle cell disease complicated only by 2 lifetime hospitalizations for vasoocclusive pain crisis. She presents with fever, cough, and increasing shortness of breath over the last 2 days. Her mother was recently diagnosed with influenza. CXR shows a right upper lobe infiltrate. Vital signs are: temperature 38.8, HR 130, RR 30, BP 110/60, O2 sat 96% on room air. Hemoglobin is 9 g/dL, which is the patient’s baseline.

What is the likely diagnosis and appropriate management?
HemeID

pemsou5_wp • September 3, 2019


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  1. Kelly September 4, 2019 - 12:17 am Reply

    D) Acute chest syndrome, admission to hospital, IV ceftriaxone and oral azithromycin
    Acute chest syndrome is the second most common reason for hospitalization of pediatric patients with sickle cell disease. It presents similar to bacterial pneumonia with cough, shortness of breath, increased respiratory work, pulmonary infiltrate, and fever. In children, upper or middle lobe involvement is most common, whereas in adults, multilobar involvement typically occurs. Hemoglobin may fall significantly below baseline. Treatment should include admission to the hospital, IV cephalosporin and oral macrolide, supplemental oxygen as needed to keep the O2 sat > 95%, blood transfusion if hemoglobin is > 1 g/dL below baseline, and exchange transfusion in rapidly progressive severe disease. What appears to be community-acquired or aspiration pneumonia should be treated as presumed acute chest syndrome in patients with sickle cell disease. Influenza is less likely to present with a focal infiltrate, and the patient must be assumed to have acute chest syndrome due to the high morbidity associated with untreated acute chest syndrome.

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