What is the Diagnosis?

A 12 y/o M with a pmh of sickle cell disease presents to the ED for evaluation of chest pain and sob x 1 day’s duration.  The child appears in mild respiratory distress, vs are normal with exception of the SpO2 of 93% on RA.  Below is the chest x-ray.  

What is the diagnosis of this patient and please describe general mgmt recommendations?

Answer: Acute Chest Syndrome


  • Etiology is likely multi-factorial: Infection, infarction (sickled cells can lead to infarction of lung tissue), and/or fat embolism
  • Dx by new infiltrate on CXR with one of the following symptoms: CP, fever, cough, sob, hypoxia 
  • Acute chest can be life threatening and there should be a low suspicion to admit patients even with lack of cxr findings

General Management Recommendations:

  1. ABC
    • High flow O2
    • Give IVFs (D51/2NS) and encourage oral intake
  2. Pain Control
  3. If CP → order CXR
  4. Labs - CBC, Retic count, Type and screen, blood cultures (if febrile)
  5. Consider broad-spectrum antibiotics → broad-spectrum abx should be given if febrile or severely ill acute chest patients
  6. Consult Hematology urgently  
  7. Consider transfusion
    • O2 Sat < 92%
    • HCT < 10-20% of baseline
    • Clinical or radiologic progression of disease
    • Situations where exchange tx may be delayed
  8. Exchange Transfusion
    • Clinical progression despite simple rx
    • Severe Hypoxemia
    • Multilobar Infiltrates

Dispo - ADMIT

Happy Studying,

Doc Roddy