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

Chest. 1999 Oct;116(4):1127-9. doi: 10.1378/chest.116.4.1127.

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.

Publication types

  • Case Reports

MeSH terms

  • Antihypertensive Agents / administration & dosage*
  • Antihypertensive Agents / adverse effects
  • Chronic Disease
  • Epoprostenol / administration & dosage*
  • Epoprostenol / adverse effects
  • Female
  • Gaucher Disease / drug therapy*
  • Humans
  • Hypertension, Pulmonary / drug therapy*
  • Infusions, Intravenous
  • Middle Aged

Substances

  • Antihypertensive Agents
  • Epoprostenol