Weisdorf S A, Freese D K, Fath J J, Tsai M Y, Cerra F B
Department of Pediatrics, University of Minnesota, Minneapolis.
J Pediatr Gastroenterol Nutr. 1987 Nov-Dec;6(6):860-4. doi: 10.1097/00005176-198711000-00007.
We measured fasting plasma amino acids in 26 children aged 6 months to 5 years with extrahepatic biliary atresia and cirrhosis and compared them with fasting values in 95 normal control children aged 4 months to 12 years. We found that the cirrhotic children had elevations of total free plasma amino acids implying reduced hepatic metabolism of amino acids and that the molar ratio of the branched chain amino acids (isoleucine, leucine, and valine) to the aromatic amino acids (phenylalanine and tyrosine) was significantly depressed. Methionine was also markedly elevated, and taurine concentrations were significantly decreased. Manipulation of the amino acid distribution in dietary protein to normalize plasma amino acids prior to orthotopic hepatic transplantation may be helpful in improving amino acid utilization.
我们测定了26名年龄在6个月至5岁的肝外胆道闭锁和肝硬化患儿的空腹血浆氨基酸水平，并将其与95名年龄在4个月至12岁的正常对照儿童的空腹值进行比较。我们发现，肝硬化患儿的血浆总游离氨基酸水平升高，这意味着肝脏对氨基酸的代谢减少，并且支链氨基酸（异亮氨酸、亮氨酸和缬氨酸）与芳香族氨基酸（苯丙氨酸和酪氨酸）的摩尔比显著降低。蛋氨酸也显著升高，而牛磺酸浓度显著降低。在原位肝移植前调整膳食蛋白质中的氨基酸分布以使血浆氨基酸正常化，可能有助于提高氨基酸利用率。
