Systemic fat embolism and pulmonary hypertension in sickle cell disease.
Castro O.
Department of Medicine, Howard University College of Medicine, Washington, DC, USA.
Systemic fat embolism, a relatively rare complication of sickle cell disease, is difficult to diagnose and it is often fatal. A high index of suspicion and early transfusion therapy may provide the best chance for recovery. Sickle cell-related pulmonary hypertension can be documented by cardiac catheterization but has no proven treatment. Patients with this complication are usually adults, have a poor prognosis, and may be considered for hydroxyurea treatment. Administration of vasodilators, anticoagulation, or oxygen may be beneficial in selected individuals.
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PMID: 8956017 [PubMed - indexed for MEDLINE]