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

Gaucher's disease is a rare disorder characterized by a deficiency of lysos omal P-glucosidase. Pulmonary: hypertension, the etiology of which is uncle ar, has: been reported to occur in association with Gaucher's disease. We r eport the ase of continuous intravenous epoprostenol (prostacyclin),which h as 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 for m of therapy for chronic pulmonary hyper tension due,to a variety of condit ions, one of which is Gaucher's disease.