Journal of the Korean Pediatric Society 1989;32(10):1377-1383.
Published online October 31, 1989.
Plasma Amino Acid Patterns in Reye's Syndrome.
Han Wook Yoo, Hee Shang Youn
Department of Pediatrics, College of Medicine, Gyeong-Sang National University, Chinju, Korea
Reye 증후군 환아의 혈장 유리 아미노산 농도
유한욱, 윤희상
경상대학교 의과대학 소아과학교실
Received: 7 January 1989   • Accepted: 5 June 1989
Abstract
The metabolic disturbances of Reye’s syndrome are many and involve carbohydrates, amino acids, free fatty acids, proteins, ammonia and acid-base balance. It has been suggested that the derangement of mitochondrial integrity by an agent or agents unknown leads to a loss of enzyme activity in many metabolic pathways in which t노e mitochondria are involoved. Of primary interests in this study is the abnormalities regarding amino acids which have been described as unique. Plasma amino acid concentrations were measured quantitatively in seven children with Reye’s syndrome, then compared with those in three healthy chidren, three patients suffering from acute hepatitis and five patients reported by Romshe et al. as Reye-like illnesses. (Fructose intolerance, ornithine transcarbamylase deficiency, systemic carnitine deficiency) The salient feature of plasma amino acid concentrations in Reye’s syndrome is significant increase in the mean concentrations of threonine, glutamate and alanine (54.6 ug/dl, 16.7 ug/dl, 89.6 ug/dl respectively), although there are wide variations among patients. Plasma amino acid patterns in Reye’s syndrome seems to be similar to those in ornithine transcarbamylase deficiency.
Key Words: Plasma Amino Acid Concentrations, Reye’s syndrome


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