The Use of Ornithine Salts of Branched-Chain Ketoacids in Portal-Systemic Encephalopathy

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Abstract
In 8 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 [day]) vs. ornithine salts of branched-chain ketoacids (34 mmol/d), both mixtures were administered orally for 7-10 days, after control periods, during a single hospitalization. Ornithine salts of branched-chain ketoacids markedly improved EEG 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, calcium salts of branched-chain ketoacids (34 mmol/d) were administered to 6 patients after control periods; only slight improvement was seen. Patients (4) received a daily dose of ornithine .alpha.-ketoglutarate containing the same quantity of ornithine; 1 did not change and 3 deteriorated rapidly. 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.