Moving towards a novel therapeutic strategy for hyperammonemia that targets glutamine metabolism
Sign Up to like & getrecommendations! Published in 2022 at "Journal of Inherited Metabolic Disease"
DOI: 10.1002/jimd.12540
Abstract: Patients with urea cycle disorders intermittently develop episodes of decompensation with hyperammonemia. Although such an episode is often associated with starvation and catabolism, its molecular basis is not fully understood. First, we attempted to elucidate… read more here.
Keywords: moving towards; novel therapeutic; therapeutic strategy; starvation ... See more keywords
Hyperammonemia-induced changes in the cerebral transcriptome and proteome.
Sign Up to like & getrecommendations! Published in 2022 at "Analytical biochemistry"
DOI: 10.1016/j.ab.2022.114548
Abstract: Molecular alterations underlying cerebral impairment in hyperammonemic disorders such as in hepatic encephalopathy (HE) are only poorly understood. Using transcriptomics and proteomics on brains of mice with systemic hyperammonemia resulting from knockout of hepatic glutamine… read more here.
Keywords: hyperammonemia induced; cerebral transcriptome; induced changes; transcriptome proteome ... See more keywords
Bioenergetic dysfunction in a zebrafish model of acute hyperammonemic decompensation
Sign Up to like & getrecommendations! Published in 2019 at "Experimental Neurology"
DOI: 10.1016/j.expneurol.2019.01.008
Abstract: &NA; Acute hyperammonemic encephalopathy is a life‐threatening manifestation of individuals with urea cycle disorders, which is associated with high mortality rates and severe neurological sequelae in survivors. Cerebral bioenergetic failure has been proposed as one… read more here.
Keywords: hyperammonemia; dysfunction; zebrafish model; model acute ... See more keywords
Peak hyperammonemia and atypical acute liver failure: The eruption of an urea cycle disorder during hyperemesis gravidarum.
Sign Up to like & getrecommendations! Published in 2017 at "Journal of hepatology"
DOI: 10.1016/j.jhep.2017.09.009
Abstract: Inborn urea cycle disorders are under-recognised metabolic causes of hyperammonemia in adults. A 28-year-old primigravida, seven weeks pregnant, affected by hyperemesis gravidarum developed acute liver injury (ALI) and then acute liver failure (ALF) in less… read more here.
Keywords: liver failure; hyperammonemia; acute liver; hyperemesis gravidarum ... See more keywords
Nifuroxazide attenuates experimentally-induced hepatic encephalopathy and the associated hyperammonemia and cJNk/caspase-8/TRAIL activation in rats.
Sign Up to like & getrecommendations! Published in 2020 at "Life sciences"
DOI: 10.1016/j.lfs.2020.117610
Abstract: Hyperammonemia is a serious metabolic disorder associating with hepatic encephalopathy (HE) which occurs secondary to several forms of liver injury ranging from simple acute liver failure (ALF) to its most serious form; cirrhosis. The resent… read more here.
Keywords: experimentally induced; caspase trail; trail; hyperammonemia ... See more keywords
Hyperammonemia compromises glutamate metabolism and reduces BDNF in the rat hippocampus
Sign Up to like & getrecommendations! Published in 2017 at "Neurotoxicology"
DOI: 10.1016/j.neuro.2017.05.006
Abstract: HIGHLIGHTSBDNF is reduced in hippocampus under ammonia toxicity.Astrocytes play an important role in BDNF secretion.Glutamine–glutamate cycle is compromised in hyperammonemia in hippocampus. ABSTRACT Ammonia is putatively the major toxin associated with hepatic encephalopathy (HE), a… read more here.
Keywords: compromises glutamate; hippocampus; hyperammonemia; hyperammonemia compromises ... See more keywords
Compound heterozygote variants: c.848A > G; p.Glu283Gly and c.890C > T; p.Ala297Val, of Isovaleric acid-CoA dehydrogenase (IVD) gene causing severe Isovaleric acidemia with hyperammonemia
Sign Up to like & getrecommendations! Published in 2022 at "Molecular Genetics and Metabolism Reports"
DOI: 10.1016/j.ymgmr.2022.100859
Abstract: With the execution of expanded newborn screen (NBS) program nationwide, it is uncommon to see severe hyperammonemia associated with isovaleric acidemia (IVA). We present a seven-day-old boy with severe IVA complicated by hyperammonemia. This child… read more here.
Keywords: compound heterozygote; isovaleric acidemia; acid; hyperammonemia ... See more keywords
An Intractable Case of Anti-N-methyl-D-aspartate Receptor Encephalitis with Prolonged Hyperammonemia
Sign Up to like & getrecommendations! Published in 2022 at "Journal of Neurosciences in Rural Practice"
DOI: 10.1055/s-0042-1744124
Abstract: Acute encephalitis is a life-threatening neurological illness that presents as a rapidly progressive encephalopathy due to inflammation of the brain.1 In the recent past, in addition to infectious causes, various autoantibody-mediated encephalitis has been identified.… read more here.
Keywords: autoimmune encephalitis; methyl aspartate; encephalitis; case ... See more keywords
Hyperammonemia in the setting of Roux-en-Y gastric bypass presenting with osmotic demyelination syndrome
Sign Up to like & getrecommendations! Published in 2021 at "Journal of Community Hospital Internal Medicine Perspectives"
DOI: 10.1080/20009666.2021.1952522
Abstract: ABSTRACT Hyperammonemia can lead to serious outcomes including brain herniation, coma and death. It is often attributed to liver disease, specifically in association with alcohol use. However, in the absence of liver pathology, it can… read more here.
Keywords: demyelination; gastric bypass; hyperammonemia; demyelination syndrome ... See more keywords
Valproic Acid–Associated Hyperammonemia
Sign Up to like & getrecommendations! Published in 2023 at "Journal of Clinical Psychopharmacology"
DOI: 10.1097/jcp.0000000000001689
Abstract: Abstract Background Hyperammonemia is an adverse effect that poses clinical uncertainty around valproic acid (VPA) use. The prevalence of symptomatic and asymptomatic hyperammonemia and its relationship to VPA concentration is not well established. There is… read more here.
Keywords: acid associated; vpa; associated hyperammonemia; valproic acid ... See more keywords
Sudden valproate-induced hyperammonemia managed with L-carnitine in a medically healthy bipolar patient
Sign Up to like & getrecommendations! Published in 2017 at "Medicine"
DOI: 10.1097/md.0000000000008117
Abstract: Rationale: Valproic Acid is a commonly used psychiatric drug primarily used as a mood stabilizer. Mild hyperammonemia is a Valproic Acid common adverse effect. This report presents an example of treated hyperammonemia on Valproic acid… read more here.
Keywords: managed carnitine; hyperammonemia; valproic acid; therapy ... See more keywords