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Case Report
A Case of Ornithine Transcarbamylase Deficiency Successfully Treated with Protein Restriction and Living Related Liver Transplantation
Bong Seong Kim, Kyung Mo Kim, Han-Wook Yoo, Sung Gyu Lee
Clin Exp Pediatr. 1999;42(6):868-873.   Published online June 15, 1999
Ornithine transcarbamylase deficiency(OTCD), the most common inborn error of the urea cycle, is inherited in X-linked manner. In affected hemizygote males, OTCD manifests hyperammonemic coma that often leads to death during the newborn period. Our patient was at high risk for inborn error of urea cycle metabolism, since his two elder brothers died a few days after birth due to...


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