Herlong H F, Maddrey W C, Walser M
Ann Intern Med. 1980 Oct;93(4):545-50. doi: 10.7326/0003-4819-93-4-545.
In eight patients with chronic portal-systemic encephalopathy who were symptomatic despite protein restriction and lactulose, a double-blind crossover comparison was conducted of branched-chain amino acids (68 mmol/d) versus ornithine salts of branched-chain ketoacids (34 mmol/d), both mixtures being administered orally for 7 to 10 days, after control periods, during a single hospitalization. Ornithine salts of branched-chain ketoacids markedly improved electroencephalographic abnormalities and clinical grade of encephalopathy; branched-chain amino acids had significantly lesser effects, which were of borderline statistical significance. To ascertain whether ornithine or branched-chain ketoacids were responsible for the improvement observed, we administered to six patients calcium salts of branched-chain ketoacids (34 mmol/d) after control periods; only slight improvement was seen.. Four patients received a daily dose of ornithine alpha-ketoglutarate containing the same quantity of ornithine; one did not change and three deteriorated rapidly. We conclude that the combination of ornithine and branched-chain ketoacids improves chronic portal-systemic encephalopathy more than its components given separately and more than branched-chain amino acids at twice the molar dose.
在8例尽管限制蛋白质摄入并使用乳果糖仍有症状的慢性门体性脑病患者中，进行了一项双盲交叉比较，比较支链氨基酸（68 mmol/d）与支链酮酸鸟氨酸盐（34 mmol/d），两种混合物均在单次住院的对照期后口服给药7至10天。支链酮酸鸟氨酸盐显著改善了脑电图异常和脑病临床分级；支链氨基酸的效果明显较差，具有边缘统计学意义。为确定是鸟氨酸还是支链酮酸导致了观察到的改善，我们在对照期后给6例患者服用支链酮酸钙盐（34 mmol/d）；仅见轻微改善。4例患者每日服用含等量鸟氨酸的鸟氨酸α-酮戊二酸；1例无变化，3例迅速恶化。我们得出结论，鸟氨酸和支链酮酸的组合比单独给予其成分以及比摩尔剂量两倍的支链氨基酸更能改善慢性门体性脑病。
