... that inherited deficiency of OTC should be included in the differential diagnosis of hyperammonemic states in adult male patients. 11 8 2017 Hyperammonemia Ornithine transcarbamylase deficiency Adolescence and adulthood, male Enzyme 1990;43:160-168 1607211 © 1990 S. Karger AG, Basel 0013-9432...

... Hyperammonemia Encephalopathy Enzyme 27:45-51 (1982) 1612658 Serum Ornithine Carbamyl Transferase Activities in Reye s Syndrome1 Devendra R. Deshmukh, Judith W. Rittenhouse, Merle Mason, Joseph V. Baublis f Departments of Biological Chemistry and Pediatrics and Communicable Diseases, University of Michigan...

...Devendra R. Deshmukh; Walter Kao; Merle Mason; Hunein F. Maassab; Joseph V. Baublist Young male ferrets developed hyperammonemia and encephalopathy soon after eating a diet lacking arginine. Because of this characteristic and their known susceptibility to influenza infections, they were further...

...C. Bachmann A rapid recognition of congenital hyperammonemia, a clear diagnostic workup and institution of a combined treatment without delay, by restriction of nitrogen supply, adequate caloric supply, substitution of missing metabolites, and use of alternate routes of nitrogen excretion will help...

... hyperammonemic coma, although ongoing accumulation of urea cycle intermediates may also play a role. Early recognition and treatment are critical if a good outcome is to be possible. Hyperammonemia Ammonia Inborn errors Benzoate Phenylacetate Arginine 11 8 2017 1987 Copyright / Drug...

...C. Bachmann Hyperammonemia in pediatrics can be due to a number of causes (defects of urea cycle enzymes or transport of its metabolites, organic acidurias, acyl-CoA dehydrogenase or carnitine deficiency, liver bypass or nonspecific insufficiency) requiring differentiated rapid treatment...

...J. P. Colombo In congenital urea cycle disorders, detoxification of ammonia is impaired, leading to hyperammonemia. Ammonia is the major component causing the acute neurological disturbances. It may influence the supply of substrate and its transport at the blood-brain barrier (BBB) which results...

...J.P. Colombo; R. Herz; J. Bircher The paper considers whether shunting of portal blood around the liver and decreased total hepatic blood flow are sufficient to explain the degree of hyperammonemia occurring after portacaval shunt (PCS). For this purpose, urea-cycle enzymes,transaminases...