Successful Bilateral Lung Transplantation for Pulmonary Fibrosis Associated With the Hermansky-Pudlak Syndrome

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 st...

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Veröffentlicht in:The Journal of heart and lung transplantation 2005-10, Vol.24 (10), p.1697-1699
Hauptverfasser: Lederer, David J., Kawut, Steven M., Sonett, Joshua R., Vakiani, Efsevia, Seward, Samuel L., White, James G., Wilt, Jessie S., Marboe, Charles C., Gahl, William A., Arcasoy, Selim M.
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Sprache:eng
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Zusammenfassung: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.
ISSN:1053-2498
1557-3117
DOI:10.1016/j.healun.2004.11.015