The use of ornithine salts of branched-chain ketoacids in portal-systemic encephalopathy.
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.
D000328:Adult; D000368:Aged; D000596:Amino Acids / Q000097:blood; D000597:Amino Acids, Branched-Chain / Q000627:therapeutic use; D000641:Ammonia / Q000097:blood; D002118:Calcium / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D004311:Double-Blind Method; D004569:Electroencephalography; D005260:Female; D006501:Hepatic Encephalopathy / Q000188:drug therapy*; D006801:Humans; D007651:Keto Acids / Q000627:therapeutic use*; D007656:Ketoglutaric Acids / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009584:Nitrogen / Q000378:metabolism; D009952:Ornithine / Q000627:therapeutic use*
D000596:Amino Acids; D000597:Amino Acids, Branched-Chain; D007651:Keto Acids; D007656:Ketoglutaric Acids; D000641:Ammonia; D009952:Ornithine; D009584:Nitrogen; D002118:Calcium