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(Chest. 1999;116:1127-1129.)
© 1999 American College of Chest Physicians

Continuous Intravenous Epoprostenol Therapy for Pulmonary Hypertension in Gaucher's Disease*

Alan E. Bakst, MD; Sean P. Gaine, MD, FCCP and Lewis J. Rubin, MD, FCCP

* From the Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Maryland, Baltimore, MD.

Correspondence to: Lewis J. Rubin, MD, FCCP, Division of Pulmonary and Critical Care Medicine, University of California, San Diego, 200 W. Arbor Dr, San Diego, CA 92103

Gaucher's disease is a rare disorder characterized by a deficiency of lysosomal ß-glucosidase. Pulmonary hypertension, the etiology of which is unclear, has been reported to occur in association with Gaucher's disease. We report the use of continuous intravenous epoprostenol (prostacyclin), which has been used to treat other forms of pulmonary hypertension, in a patient with pulmonary hypertension associated with Gaucher's disease. Although its mechanism of action remains unknown, epoprostenol may be an effective form of therapy for chronic pulmonary hypertension due to a variety of conditions, one of which is Gaucher's disease.

Key Words: epoprostenol • Gaucher's disease • pulmonary hypertension




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