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PubMed1983

Activity of the glycine cleavage system in hyperammonemia treated with benzoate.

We investigated levels of the glycine cleavage system in livers of spf-fur mutant mice with ornithine transcarbamylase (OTC) deficiency treated with sodium benzoate. The activities of the glycine cleavage system in benzoate-treated spf/Y males and the control mice livers are not significantly different. We examined plasma folate level and Vit. B6 status in a patient with OTC deficiency during the therapy with benzoate. Plasma folate level and vitamin B6 status during the therapy period and during control period are not different and these data were within normal ranges. The observation suggests that the glycine cleavage system is not the candidate for the increase of the de novo synthesis of glycine in hyperammonemic patients treated with benzoate.

Ammonia↗
PubMedDate not supplied

[The hyperammonemia of exercise; value of automatic measurement].

Ammonia blood level (NH3) was measured during maximum exercise performed on cycloergometer, in 11 patients. NH3 measurements through an automatic chemical method (whole blood) were compared to those of the enzymatic reference method (plasma). Automatic analysis made it possible to quickly obtain [NH3] values, that were highly correlated with those of the enzymatic method (P < 0.001). Plasma [NH3] may be calculated from the following equation, where it is expressed versus [NH3] obtained with the automatic method (c anal): [NH3] mumol.l-1 = 0.795. [c] anal.microgram.dl-1 + 5.446.

Ammonia↗
PubMed1991

[Therapy of hyperammonemia in carbamyl phosphate synthase deficiency with peritoneal dialysis and venovenous hemofiltration].

Inborn errors of metabolism with hyperammonaemia cause emergency situations with unconsciousness, convulsion, hyperpnoea and hyperpyrexia. Therefore hyperammonaemia has to be treated immediately after diagnosis to avoid irreversible damage. Two newborns with carbamylphosphates synthetase deficiency are described. Both, continuous peritoneal dialysis and venovenous haemofiltration have proved to be effective methods to reduce serum ammonia concentration to values of less than 300 mu/l. Because of the severity of the enzyme defect in both cases, (carbamylphosphate synthetase was not detectable in liver tissue), treatment finally had to be stopped, and both patients died.

Amino Acid Metabolism, Inborn Errors↗