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Archives of Pediatrics & Adolescent Medicine, 1967
AN UNDERSTANDING of the nature of the underlying biochemical disorder in hyperammonemia is essential to a rational approach to therapy of this condition. Three types of hereditary enzyme defect in the biosynthesis of urea have been characterized in recent years (Table 1): arginosuccinic aciduria (reaction 4), citrullinuria (reaction 3), and ...
B, Levin, A, Russell
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AN UNDERSTANDING of the nature of the underlying biochemical disorder in hyperammonemia is essential to a rational approach to therapy of this condition. Three types of hereditary enzyme defect in the biosynthesis of urea have been characterized in recent years (Table 1): arginosuccinic aciduria (reaction 4), citrullinuria (reaction 3), and ...
B, Levin, A, Russell
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Pharmacotherapy for hyperammonemia
Expert Opinion on Pharmacotherapy, 2014Hepatic encephalopathy (HE) is a serious neuropsychiatric complication that is seen in patients with liver failure. The pathogenesis of HE is not entirely understood, but several hypotheses have emerged and persisted during the years. Despite the many prevalent hypotheses, most of the existing evidence point to ammonia as the main culprit behind ...
Anna, Hadjihambi +2 more
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Current Problems in Pediatrics, 1984
A symptomatic elevation in plasma ammonium concentration, termed hyperammonemia, is associated with numerous congenital and acquired conditions (Table 11). In some cases, such as urea cycle disorders, ammonia is the principal toxin. In other instances, such as portal systemic encephalopathy, it is but one of a number of metabolic disturbances, However,
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A symptomatic elevation in plasma ammonium concentration, termed hyperammonemia, is associated with numerous congenital and acquired conditions (Table 11). In some cases, such as urea cycle disorders, ammonia is the principal toxin. In other instances, such as portal systemic encephalopathy, it is but one of a number of metabolic disturbances, However,
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Clinical Chemistry and Laboratory Medicine, 2002
Hyperammonemia is mainly found in hepatic encephalopathy and in genetic defects of the urea cycle or other pathways of the intermediary metabolism. Clinically a difference has to be made between chronic moderate hyperammonemia and acutely increased concentrations. Pathogenetic mechanisms of ammonia toxicity to the brain are partly unraveled.
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Hyperammonemia is mainly found in hepatic encephalopathy and in genetic defects of the urea cycle or other pathways of the intermediary metabolism. Clinically a difference has to be made between chronic moderate hyperammonemia and acutely increased concentrations. Pathogenetic mechanisms of ammonia toxicity to the brain are partly unraveled.
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A case of transient hyperammonemia in the newborn transient neonatal hyperammonemia
The Journal of Maternal-Fetal & Neonatal Medicine, 2009A case of transient hyperammonemia of the newborn (THAN) is described in this paper. THAN is the disorder that is much more frequently present than diagnosed. Therefore, it is necessary to estimate the serum ammonia level in every preterm newborn infant, who develops the signs of respiratory distress syndrome in the first hours of life, along with the ...
Vesna D, Stojanovic +4 more
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Hyperammonemia in acetaminophen toxicity
Clinical Toxicology, 2021Acetaminophen-induced hepatotoxicity can result in hyperammonemia, but it is not clear if elevated ammonia concentrations predict encephalopathy.We retrospectively studied patients with acetaminophen toxicity at a liver transplant center over 8 years (January 1, 2010-December 31, 2017), who developed hepatotoxicity (AST and/or ALT >1000 IU/L) or ...
Ryan T. Marino, Alexander M. Sidlak
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Hyperammonemia: The Silent Killer
Southern Medical Journal, 1993Nitrogen, derived from breakdown of dietary amino acids as ammonia, is normally converted to urea and excreted. Impairment in the conversion process (called the urea cycle) can occur, either as a consequence of primary genetic defects or through secondary suppression of enzyme activity.
D E, Miga, K S, Roth
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Hyperammonemia in Alzheimer's disease
American Journal of Psychiatry, 1985Postprandial blood ammonia levels were significantly higher in 22 patients with Alzheimer's disease than in 37 control subjects. In the Alzheimer group, fasting blood ammonia levels were significantly higher in patients whose EEGs showed triphasic waves than in patients without this change.
M, Fisman +5 more
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Postictal transient hyperammonemia
The American Journal of Emergency Medicine, 2008We report cases of several patients who were sent to our hospital because of seizure attacks. Transient hyperammonemia was noted. Many biochemistry abnormalities, such as prolactin and creatine kinase, may be found after an episode of seizures, but hyperammonemia seems to not have been reported before.
Kuan-Ting, Liu, Chen-San, Su
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Hyperammonemia in the Neonate with Hypoxia
1982Inherited defects in enzymes of the urea cycle and those in metabolism of several organic acids and some amino acids can cause neonatal hyperammonemia (Shih, 1978). Ballard et al. (1978) described five newborns with respiratory distress who developed hyperammonemia, but exhibited normal activities of urea cycle enzymes.
Y, Sakaguchi +4 more
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