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At least 577 records · Page 32Linked to original sources
PubMed1987

Pharmacologic amino acid acylation in the acute hyperammonemia of propionic acidemia.

An infant with propionic acidemia presented at one month of age with hyperammonemic coma. Treatment by two double-volume exchange transfusions did not have an appreciable effect, but hemodialysis led to a substantial reduction of the serum concentration of ammonia on two occasions. Nevertheless, continued therapy with sodium benzoate, arginine-HCl, carnitine and lactulose did not have any observable effect on the blood concentration of ammonia. Treatment with sodium phenylacetate was followed by a reduction in serum concentrations of ammonia to normal levels which were maintained. These observations demonstrate the dramatic reduction in serum concentrations of ammonia that may be obtained in patients with organic acidemia by hemodialysis. They suggest that pharmacologic acylation therapy with phenylacetate may be of lasting benefit in the management of this complication.

Acylation↗
PubMed1985

Transient hyperammonemia during aging in ornithine transcarbamylase-deficient, sparse-fur mice.

Ornithine transcarbamylase [EC 2.1.3.3] (OTC) deficient, sparse-fur (spf) mice are considered to be a good model of human OTC deficiencies. We measured the blood ammonia concentration in spf mice. It was not always at a high level. It was high only during weaning, then gradually decreased and finally fell to a normal level. A similar phenomenon was observed in the change of hepatic ammonia concentration. Excretion of urinary orotate in spf mice was 15-20 times higher than in control mice just after weaning. It also gradually decreased during aging, but remained at a high level compared with adult control mice. OTC activity at pH 7.7 in the liver of spf mice increased significantly between the weaning period and 30-40 days old, when blood ammonia was falling to a normal level.

Aging↗
PubMed1977

[Hyperammonemia during parenteral nutrition of intensive-care patients].

Metabolic features of parenteral feeding with conventional amino acid solutions were examined in 47 patients over a long period. 30 patients were kept alive by artificial respiration. The metabolic parameters ammonium, blood urea nitrogen, GOT, alkaline phosphatase were carried out, in 6 patients the pattern of amino acids was analysed. All patients showed a significant increase of ammonium during the course of parenteral feeding. The amino acids demonstrated pattern of imbalance. The other other parameters were not changed significantly. Traumatic, hypoxic or toxic liver damage might influence the reduction of liver function.

Adult↗