deamino-arginine-vasopressin and Pulmonary-Fibrosis

Hermansky-Pudlak syndrome (HPS) is a genetic disorder characterized by oculocutaneous albinism, a bleeding diathesis, and in a subset of patients, pulmonary fibrosis. Lung transplantation, the only curative therapy for pulmonary fibrosis, has not been previously reported as a successful treatment strategy for patients with HPS because the bleeding diathesis was thought to contraindicate major thoracic surgery. We successfully performed bilateral sequential lung transplantation in a patient with pulmonary fibrosis and HPS after transfusion of 6 units of platelets. Lung transplantation is a viable therapeutic option in patients with pulmonary fibrosis and only a mild bleeding diathesis associated with HPS.

Topics: Adult; Blood Platelet Disorders; Deamino Arginine Vasopressin; Hemorrhagic Disorders; Hemostatics; Hermanski-Pudlak Syndrome; Humans; Lung Transplantation; Male; Membrane Proteins; Platelet Transfusion; Pulmonary Fibrosis; Treatment Outcome

Topics: Administration, Intranasal; Adult; Arginine Vasopressin; Asthma; Deamino Arginine Vasopressin; Diabetes Insipidus; Drug Hypersensitivity; Humans; Male; Pulmonary Fibrosis; Vasopressins