New England Journal of Medicine · 1981 · 57 citations · 9 references
MedicineInfectious DiseasesNeurologyHepatologyNeurourologyRenal PathophysiologyAmino Acid Metabolism DisordersHepatic EncephalopathyLiver DiseaseNeurogenic BladderUrea CycleUrea Cycle DisordersBacterial Metabolite ProductionEnzymesInborn Errors Of Urea Cycle MetabolismLiver Enzyme Abnormalities
THE contribution of ammonia to the pathogenesis of hepatic encephalopathy has been widely investigated.1 , 2 Hyperammonemia and cerebral dysfunction in the absence of liver disease and portal hypertension have been reported in patients with acquired3 or congenital4 , 5 deficiency of hepatic enzymes of the Krebs urea cycle and in patients who have had ureterosigmoidostomy.6 7 8 9 However, in the latter group coexistent liver disease was present and could have contributed to encephalopathy.6 7 8 9 We report the case of a patient who had recurrent hyperammonemic encephalopathy despite adequate urea-cycle enzymes and normal liver structure. The source of the elevated blood ammonia was excessive bacterial production of . . .
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Hepatic Encephalopathy: Current Status
Steven Schenker, Kerry J. Breen, Anastacio M. Hoyumpa · Gastroenterology · 1974 · 144 citations
Inherited Hyperammonemic Syndromes
Y. Edward Hsia · Gastroenterology · 1974 · 87 citations