A case of adult-onset type II citrullinemia induced by hospital diet
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : Akira Ueda Kazuhiko Okada Terumi Takahara Keiichi Iwasa Kazuko Shinagawa Akio Entani Syuji Inatsuchi Takayuki Ando Haruka Fujinami
- چاپ و سال / کشور: 2011
Description
A 47-year-old Japanese man was first admitted to our hospital for 8 days because of an asthma attack. After discharge he changed his diet. On the 12th day after his discharge, he was re-admitted to our hospital because he exhibited transient loss of consciousness with flapping tremor. His plasma ammonia level was extremely high (245 lg/dL; normal,\90 lg/dL), suggesting hepatic encephalopathy. He underwent intravenous administration of branched-chain amino acids (Aminoleban) and oral administration of lactulose and kanamycin sulfate; however, the hyperammonemia did not improve. Analysis of the amino acids and citrin gene led to the diagnosis of adult-onset type II citrullinemia (CTLN2). Following this diagnosis, the carbohydrate content of his diet was mildly restricted. As a result, his plasma ammonia level markedly improved (ammonia, 40–60 lg/dL) and he became symptom- free without any medication. CTLN2 is a metabolic disorder characterized by increased plasma concentrations of citrulline and ammonia, which occurs by the failure of compensatory mechanisms associated with diet. Here, we report a case of a patient for whom a change in eating habits during his hospitalization disturbed his compensatory mechanism resulting in clinical CTLN2, which was reversed with an appropriate diet.
Clin J Gastroenterol (2011) 4:28–33 Received: 27 July 2010 / Accepted: 3 November 2010 / Published online: 17 December 2010 Springer 2010
