A Case of Ornithine Transcarbamylase (OTC) Deficiency
Journal of the Korean Pediatric Society
;
: 123-127, 2000.
Article
in Korean
| WPRIM
| ID: wpr-217857
ABSTRACT
OTC deficiency is an X-linked disorder in which the synthesis of urea is impaired. OTC catalyzes the synthesis of citrulline from carbamyl phosphate and ornithine. Complete or partial deficiencies of this enzyme may lead to Reye syndrome like picture such as encephalopathy, hepatic dysfunction, hyperammonemia, etc. We recently had a case that was presented as recurrent Reye syndrome, and was effectively treated with hemodialysis, arginine, sodium benzoate, etc. This report describes an experience in treating this condition with review of available literature.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Ornithine
/
Ornithine Carbamoyltransferase
/
Arginine
/
Reye Syndrome
/
Urea
/
Carbamyl Phosphate
/
Hepatic Encephalopathy
/
Renal Dialysis
/
Citrulline
/
Sodium Benzoate
Language:
Korean
Journal:
Journal of the Korean Pediatric Society
Year:
2000
Type:
Article
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