TITLE:
Anesthetic Management of a Patient with Hermansky-Pudlak Syndrome
AUTHORS:
Kelly Zach, Lopa Misra
KEYWORDS:
Hermanksy-Pudlak, Anesthesia
JOURNAL NAME:
Open Journal of Anesthesiology,
Vol.5 No.2,
February
26,
2015
ABSTRACT: Hermansky-Pudlak Syndrome (HPS) is a rare genetic condition characterized by platelet function abnormalities and oculocutaneous albinism. Other systemic manifestations of the disease include pulmonary fibrosis, granulomatous colitis, impaired renal function, and cardiomyopathy. Due to the systemic extent of the disease, HPS has multiple anesthetic concerns and requires a detailed preoperative evaluation and close perioperative monitoring.