title: Continuous intravenous epoprostenol therapy for pulmonary hypertension in Gaucher's disease.
abstract: Gaucher's disease is a rare disorder characterized by a deficiency of lysosomal beta-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.
authors.affiliation: Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Maryland, Baltimore, MD.
authors.forename: A E
authors.initials: AE
authors.lastname: Bakst
authors.forename: S P
authors.initials: SP
authors.lastname: Gaine
authors.forename: L J
authors.initials: LJ
authors.lastname: Rubin
mesh_terms: D000959:Antihypertensive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D002908:Chronic Disease; D011464:Epoprostenol / Q000008:administration & dosage* / Q000009:adverse effects; D005260:Female; D005776:Gaucher Disease / Q000188:drug therapy*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy*; D007262:Infusions, Intravenous; D008875:Middle Aged
chemical_list: D000959:Antihypertensive Agents; D011464:Epoprostenol