Continuous intravenous epoprostenol therapy for pulmonary hypertension in Gaucher's disease

Gaucher's disease is a rare disorder characterized by a deficiency of lysosomal P-glucosidase. Pulmonary: hypertension, the etiology of which is unclear, has: been reported to occur in association with Gaucher's disease. We report the ase of continuous intravenous epoprostenol (prostacyclin),which has been used to treat other forms of pulmonary hyper-tension, 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 hyper tension due,to a variety of conditions, one of which is Gaucher's disease.