An International Publisher for Academic and Scientific Journals
Author Login 
Scholars Journal of Medical Case Reports | Volume-2 | Issue-06
Anesthetic Management of a Female Patient with Ornithine Transcarbamylase Deficiency
Victor Leslie, Medhat Hannallah
Published: June 29, 2014 | 135 93
DOI: 10.36347/sjmcr.2014.v02i06.013
Pages: 391-393
Downloads
Abstract
Ornithine transcarbamylase deficiency (OTCD) is an X-linked, inherited condition within the urea cycle characterized by failure of ammonia detoxification and urea formation. This may lead to hyperammonemic encephalopathy that, if uncontrolled, results in brain injury and death. Individuals susceptible to this disorder are at risk for hyperammonemic crises if a catabolic state is precipitated. We present an adult female patient with OTCD who underwent L5-S1 transforaminal lumbar interbody fusion. Since the NPO status and the stress of surgery predisposed the body to a catabolic state, the anesthetic management of this patient aimed at avoiding conditions that could stimulate protein catabolism.