Yudkoff M, Blazer-Yost B, Cohn R, Segal S
Am J Clin Nutr. 1979 Feb;32(2):282-5. doi: 10.1093/ajcn/32.2.282.
The ratio of plasma alpha-aminobutyric acid to plasma leucine that has been proposed as a specific biochemical marker of alcoholic liver injury, was measured in normal children an children with a variety of metablic disorders. The ratio was found to be lower in children that in adults. It was elevated in children with Reye's syndrome, tyrosinemia, homocystinuria, nonketotic hyperglycinemia, and ornithine transcarbamylase deficiency. The results indicate that an elevated ratio is not specific for adults with alcoholic liver injury and that the ratio may be high even without the presence of clinically apparent hepatic disease. An altered ratio may reflect the presence of an inherent metabolic defect.
有人提出,血浆α-氨基丁酸与血浆亮氨酸的比值可作为酒精性肝损伤的一种特异性生化标志物。我们对正常儿童和患有各种代谢紊乱的儿童进行了该比值的测定。结果发现,儿童的该比值低于成人。患有瑞氏综合征、酪氨酸血症、同型胱氨酸尿症、非酮症高甘氨酸血症和鸟氨酸转氨甲酰酶缺乏症的儿童,其该比值升高。结果表明,该比值升高并非酒精性肝损伤成人患者所特有,而且即使没有明显的临床肝病,该比值也可能升高。比值改变可能反映存在内在的代谢缺陷。