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Case Report

Min Ho Hwang, Bo Sun Kim, Dong Hwan Lee

Clin Exp Pediatr. 2001;44(10):1206-1210. Published online October 15, 2001

Hyperornithinemia-hyperammonemia-homocitrullinuria(HHH) syndrome is a rare autosomal recessive disorder caused by a defect in the urea cycle. Protein intolerance, mental retardation, seizure, ataxia, and stupor are characteristic symptoms. Patients showing these symptoms may also present symptoms of acute hepatic disease at the same time. When fed with a high protein diet, they may refuse to eat, vomit, become lethargic, or go...

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