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Case of the Week 296



What's the most likely diagnosis?

  • 27 year-old male with sickle cell disease, cough and fever

  1. Pneumothorax
  2. Pericardial effusion
  3. Acute Chest Syndrome
  4. Dislocated shoulder
  5. Congestive heart failure

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Answer:

 


3. Acute Chest Syndrome

 

 

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Acute Chest Syndrome


General Considerations

  • Leading cause of death amongst patients with sickle cell disease
  • Syndrome is characterized by
    • New airspace disease on chest x-ray, and one or more of the following:
    • Fever (variable)
    • Cough
    • Sputum production
    • Shortness of breath
    • Hypoxia
  • Exact cause is unknown
  • Most often affects those with homozygous sickle cell and sickle cell-beta thalassemia genotypes
  • More common in the winter, in younger individuals, and after surgery
  • More severe in adolescents and adults (>20) than in children

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