Medical Subject Headings

Last uploaded: March 22, 2026
Id http://purl.bioontology.org/ontology/MESH/D020163
http://purl.bioontology.org/ontology/MESH/D020163
Preferred Name

Ornithine Carbamoyltransferase Deficiency Disease

Definitions
An inherited urea cycle disorder associated with deficiency of the enzyme ORNITHINE CARBAMOYLTRANSFERASE, transmitted as an X-linked trait and featuring elevations of amino acids and ammonia in the serum. Clinical features, which are more prominent in males, include seizures, behavioral alterations, episodic vomiting, lethargy, and coma. (Menkes, Textbook of Child Neurology, 5th ed, pp49-50)
Synonyms
Ornithine Carbamoyltransferase Deficiency
Ornithine Transcarbamylase Deficiencies
Ornithine Transcarbamylase Deficiency
Deficiencies, OTC
Deficiency, Ornithine Transcarbamylase
OTC Deficiency
Deficiency Disease, Ornithine Transcarbamylase
Ornithine Transcarbamylase Deficiency, Hyperammonemia Due To
OTC Deficiencies
Deficiency, OTC
Ornithine Transcarbamylase Deficiency Disease
Deficiency Disease, Ornithine Carbamoyltransferase
Deficiencies, Ornithine Transcarbamylase
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Type http://www.w3.org/2002/07/owl#Class
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