Audience
Emergency medicine residents and medical students on emergency medicine rotations
Introduction
Acute chest syndrome is a life-threatening, potentially catastrophic complication of sickle cell disease.
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It occurs in approximately 50% of patients with sickle cell disease, with up to 13% all-cause mortality.
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Most common in children aged 2–4, up to 80% of patients with a prior diagnosis of acute chest syndrome will have recurrence of this syndrome.
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Diagnostic criteria include a new infiltrate on pulmonary imaging combined with any of the following: fever > 38.5°C (101.3°F), cough, wheezing, hypoxemia (PaO2 < 60 mm Hg), tachypnea, or chest pain.
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The pathophysiology of acute chest syndrome involves vaso-occlusion in pulmonary vessels resulting in hypoxia, release of inflammatory mediators, acidosis, and infarction of lung tissue. The most common precipitants are infections (viral or bacterial), rib infarction, and fat emboli.
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Patients commonly present with fever, dyspnea, cough, chills, chest pain, or hemoptysis. Diagnosis is made through physical exam, blood work, and chest imaging.
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Chest radiograph is considered the gold standard for imaging modality.
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Management of acute chest syndrome includes hydration with IV crystalloid solutions, antibiotics, judicious analgesia, oxygen, and, in severe cases, transfusion.
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Emergency medicine practitioners should keep acute chest syndrome as a cannot miss, high consequence differential diagnosis for all patients with sickle cell disease presenting to the Emergency Department.
Educational Objectives
At the end of this oral board session, examinees will: 1) demonstrate the ability to obtain a complete medical history; 2) demonstrate the ability to perform a detailed physical examination in a patient with respiratory distress; 3) identify a patient with respiratory distress and hypoxia and manage appropriately (administer oxygen, place patient on monitor); 4) investigate the broad differential diagnoses which include acute chest syndrome, pneumonia, acute coronary syndrome, acute congestive heart failure, acute aortic dissection and acute pulmonary embolism; 5) list the appropriate laboratory and imaging studies to differentiate acute chest syndrome from other diagnoses (complete blood count, comprehensive metabolic panel, brain natriuretic peptide (BNP), lactic acid, procalcitonin, EKG, troponin level, d-dimer, chest radiograph); 6) identify a patient with acute chest syndrome and manage appropriately (administer intravenous pain medications, administer antibiotics after obtaining blood cultures, emergent consultation w...