Intraoperative Adrenal Insufficiency in a Patient with Prader-Willi Syndrome | Zendy

David W. Barbara | Zendy; James D. Han | Zendy; William R. Hartman | Zendy

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Intraoperative Adrenal Insufficiency in a Patient with Prader-Willi Syndrome

Author(s) -

David W. Barbara,

James D. Han,

William R. Hartman

Publication year - 2012

Publication title -

journal of clinical medicine research

Language(s) - English

Resource type - Journals

eISSN - 1918-3011

pISSN - 1918-3003

DOI - 10.4021/jocmr1039w

Subject(s) - medicine , adrenal insufficiency , primary adrenal insufficiency , asymptomatic , perioperative , morbid obesity , pediatrics , adrenal disorder , hydrocortisone , sleep disordered breathing , genetic disorder , obesity , obstructive sleep apnea , surgery , weight loss , insulin resistance , disease , glucose homeostasis

Prader-Willi syndrome (PW) is a rare genetic disorder with multi-organ system involvement. These patients present many perioperative challenges including sleep-related breathing disorders, morbid obesity, thick salivary secretions, mental retardation, and difficult intravenous access. PW has been suggested to be associated with central adrenal insufficiency. We report a novel case of persistent severe hypotension from previously undiagnosed and asymptomatic adrenal insufficiency in a pediatric patient with Prader-Willi syndrome during spine surgery that resolved upon treatment with hydrocortisone.

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