hyperammonemia


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hyperammonemia

 [hi″per-am″mo-ne´me-ah]
elevated levels of ammonia or its compounds in the blood. A congenital form occurs in two types: Type 1, due to deficiency of the enzyme ornithine carbamoyltransferase, is marked by vomiting, lethargy, coma, and hepatomegaly; symptoms are aggravated by protein ingestion. Type 2, due to deficiency of the enzyme carbamoyl phosphate synthetase (ammonia), is marked by vomiting, lethargy, and flaccidity and by elevated plasma and urinary levels of glycine. Hyperammonemia may also occur in nongenetic diseases such as severe liver disease.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

am·mo·ne·mi·a

, ammoniemia (am'ō-nē'mē-ă, am'ō-ne-ē'mē-ă),
The presence of ammonia or some of its compounds in the blood, thought to be formed from the decomposition of urea; it usually results in subnormal temperature, weak pulse, gastroenteric symptoms, and coma.
Synonym(s): hyperammonemia
[ammonia + G. haima, blood]
Farlex Partner Medical Dictionary © Farlex 2012

hyperammonemia

Metabolism A heterogeneous group of five largely AR inborn errors of metabolism; each has a defect in a urea cycle enzyme–arginase, argininosuccinase, argininosuccinic acid synthetase, carbamyl phosphate synthetase, ornithine transcarbamylase; all begin in late infancy or childhood, except arginase deficiency, which is neonatal Clinical Accumulation of urea precursors–eg, ammonia, glutamine causes progressive lethargy, hyperthermia, apnea, hyperammonemia Diagnosis may be established in utero by restriction fragment–RFLP analysis Treatment Restrict dietary protein; activate alternate pathways of waste nitrogen excretion–eg, sodium benzoate or dietary supplementation with arginine
McGraw-Hill Concise Dictionary of Modern Medicine. © 2002 by The McGraw-Hill Companies, Inc.
References in periodicals archive ?
Serious adverse events included hypersensitivity and hyperammonemia, which were infrequent, managed with standard treatment and did not lead to any patient discontinuations.
Synthetic biotic medicines are microbes genetically engineered to counter the biology that drives diseases such as hyperammonemia and forms of cancer.
Rifaximin in Subjects with Hepatic Cirrhosis and a History of Prior Episodes of Hepatic Encephalopathy.' The study will include adult men and non-pregnant women with hepatic cirrhosis who have a history of at least two or more documented episodes of HE in the last 12 months - with one in the last six months - and who present with hyperammonemia at the time of screening.
Synlogic's two lead programmes, SYNB1020 and SYNB1618, are orally administered and target hyperammonemia as a result of liver damage or genetic disease, and phenylketonuria, respectively.
In case reports of hyperammonemia due to valproic acid, previously healthy patients experience confusion, lethargy, and eventual coma in the context of elevated serum ammonia levels; these symptoms resolved upon discontinuing valproic acid.
The usual toxic effects after an acute valproate overdose are: central nervous system depression, gastro-enteral effects, pancreatitis, metabolic acidosis with a high anionic gap, dyselectrolytemia and hyperammonemia (1,7,9).
Previously reported manifestations of cblD disease included developmental delay, dystonia, epilepsy, seizures, hyperammonemia, metabolic dysequilibrium, etc.[1] The patient in this study was similar to one patient in the report of Miousse et al .;[4] both of patients were admitted to hospital due to vomiting and suffered from metabolic acidosis before the diagnosis of cblD disease, and they were both responsive to Vitamin B12.
Metabolic tests were analyzed for inborn error of metabolism due to metabolic acidosis, hypoketotic hypoglycemia, hyperammonemia, and elevated liver enzymes.
He had hepatitis C and alcohol-related cirrhosis with a Model for End Stage Liver Disease score of 17, Child-Pugh score of A6, and Westhaven grade 2 encephalopahy with hyperammonemia (240 mcmol/L) despite standard medical therapy.
International studies on zinc supplementation therapy in patients having cirrhosis of liver have revealed to improve hyperammonemia and hepatic encephalopathy due to enhancement in protein metabolism.14,15
Metabolic acidosis, anemia, hyperammonemia, lactic acidosis and hyperhomocytenemia were common biochemical findings (Table).
Consciousness disorder associated with valproate-induced hyperammonemia also occurs and can lead to coma or death in severe cases (2), but there has been no published report about relationship between chronic pain and consciousness disorder associated with valproate-induced hyperammonemia.