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

H. FRANKLIN HERLONG, M.D.; WILLIS C. MADDREY, M.D.; and MACKENZIE WALSER, M.D.
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▸Requests for reprints should be addressed to Willis C. Maddrey, M.D.; Department of Medicine, The Johns Hopkins Hospital, 600 North Wolfe Street; Baltimore, MD 21205.


Baltimore, Maryland


© 1980 American College of PhysiciansAmerican College of Physicians


Ann Intern Med. 1980;93(4):545-550. doi:10.7326/0003-4819-93-4-545
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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 alphaketoglutarate 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.

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