Watanabe A, Takesue A, Higashi T, Nagashima N
Acta Hepatogastroenterol (Stuttg). 1979 Oct;26(5):346-57.
Encephalopathic patients with cirrhosis of the liver consistently showed elevated levels of the aromatic amino acids, phenylalanine, tyrosine and free tryptophan as well as methionine in serum, whereas levels of the branched chain amino acids, valine, leucine and isoleucine, were depressed. Comatose patients with fulminant hepatitis had markedly elevated levels of all amino acids, the results being greatly different from those of cirrhotic patients. Molar ratios of (valine + leucine + isoleucine)/(phenylalanine + tyrosine) decreased both in cirrhotics with and without encephalopathy and in cases with fulminant hepatitis. Infusion of a commercially available L-amino acid solution in a cirrhotic patient induced a strikingly abnormal aminogram documented in hepatic encephalopathy. Therefore, effects of branched chain amino acid infusion on the deranged amino acid pattern were primarily studied for the purpose of improvement in hepatic encephalopathy by normalization of serum amino acid patterns. Elevated levels of the aromatic amino acids and methionine could be apparently depressed in a cirrhotic patient by this type of infusion but not in a case of fulminant hepatitis probably because of the poor utilization of these amino acids in severely impaired liver.
肝硬化性脑病患者血清中芳香族氨基酸、苯丙氨酸、酪氨酸、游离色氨酸以及蛋氨酸水平持续升高,而支链氨基酸缬氨酸、亮氨酸和异亮氨酸水平降低。暴发性肝炎昏迷患者所有氨基酸水平均显著升高,结果与肝硬化患者差异极大。无论有无肝性脑病的肝硬化患者以及暴发性肝炎患者,(缬氨酸+亮氨酸+异亮氨酸)/(苯丙氨酸+酪氨酸)的摩尔比均降低。给一名肝硬化患者输注市售L-氨基酸溶液会诱发肝性脑病中记录的明显异常的氨基酸谱。因此,主要研究了输注支链氨基酸对紊乱氨基酸模式的影响,目的是通过使血清氨基酸模式正常化来改善肝性脑病。通过这种输注方式,肝硬化患者血清中升高的芳香族氨基酸和蛋氨酸水平可明显降低,但暴发性肝炎患者则不然,这可能是因为在严重受损的肝脏中这些氨基酸利用不佳。