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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
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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.