Clinical practice: the management of hyperammonemia (Q37821816)

23 August 2017

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title

Clinical practice: the management of hyperammonemia (English)

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23 August 2017

Johannes Häberle

1

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23 August 2017

17 December 2010

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European Journal of Pediatrics

23 August 2017

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23 August 2017

170

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23 August 2017

1

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23 August 2017

21-34

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https://scigraph.springernature.com/pub.10.1007/s00431-010-1369-2

23 August 2017

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cites work

Long-term outcome of patients with urea cycle disorders and the question of neonatal screening

1 reference

https://api.crossref.org/works/10.1007%2FS00431-010-1369-2

Magnetic resonance spectroscopy shows increased brain glutamine in ornithine carbamoyl transferase deficiency.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00431-010-1369-2

Valproate-induced hyperammonemic encephalopathy in the presence of topiramate

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00431-010-1369-2

Nitrogen metabolism in liver: structural and functional organization and physiological relevance

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS00431-010-1369-2

The biochemical and molecular spectrum of ornithine transcarbamylase deficiency.

21 January 2018

1 reference

12 December 2020

Neurological outcome of patients with ornithine carbamoyltransferase deficiency

1 reference

12 December 2020

1H MRS identifies symptomatic and asymptomatic subjects with partial ornithine transcarbamylase deficiency

1 reference

12 December 2020

Emergency treatment of neonatal hyperammonaemic coma with mild systemic hypothermia

1 reference

12 December 2020

Survival after treatment with phenylacetate and benzoate for urea-cycle disorders

1 reference

12 December 2020

Ammonia toxicity and its prevention in inherited defects of the urea cycle

1 reference

12 December 2020

Cross-sectional multicenter study of patients with urea cycle disorders in the United States

1 reference

12 December 2020

Hyperammonemic encephalopathy

1 reference

12 December 2020

New insights in nutritional management and amino acid supplementation in urea cycle disorders

1 reference

12 December 2020

Alternative pathway therapy for urea cycle disorders.

1 reference

12 December 2020

Metabolism and recycling of urea in man

1 reference

12 December 2020

Urea cycle disorders: diagnosis, pathophysiology, and therapy.

1 reference

12 December 2020

Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter

1 reference

12 December 2020

Neurobiology of ammonia.

1 reference

12 December 2020

Glutamine: a Trojan horse in ammonia neurotoxicity

1 reference

12 December 2020

Paradigm shift in neuroprotection by NMDA receptor blockade: Memantine and beyond

1 reference

12 December 2020

Effect of alternative pathway therapy on branched chain amino acid metabolism in urea cycle disorder patients

1 reference

12 December 2020

Neonatal hyperammonemia: the N-carbamoyl-L-glutamic acid test

1 reference

12 December 2020

Diffusion tensor imaging detects areas of abnormal white matter microstructure in patients with partial ornithine transcarbamylase deficiency

1 reference

12 December 2020

Hyperammonemia and coma without hepatic dysfunction induced by valproate therapy

1 reference

12 December 2020

Biochemistry and physiology of brain ammonia

1 reference

12 December 2020

Profiling of astrocyte properties in the hyperammonaemic brain: shedding new light on the pathophysiology of the brain damage in hyperammonaemia

1 reference

12 December 2020

Hyperammonaemic encephalopathy in a 13-year-old boy.

1 reference

12 December 2020

N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers

1 reference

12 December 2020

Nitrogen metabolism and ornithine cycle function

1 reference

12 December 2020

Glutamine utilization by the small intestine.

1 reference

12 December 2020

Blood levels of ammonia and nitrogen scavenging amino acids in patients with inherited hyperammonemia

1 reference

12 December 2020

Preanalytical increase of ammonia in blood specimens from healthy subjects.

1 reference

12 December 2020

Measurement of ammonia in blood

1 reference

12 December 2020

Outcome and survival of 88 patients with urea cycle disorders: a retrospective evaluation

1 reference

12 December 2020

Glutamine metabolism in endothelial cells: ornithine synthesis from glutamine via pyrroline-5-carboxylate synthase

1 reference

12 December 2020

Plasma ammonia concentrations in newborns and children

1 reference

12 December 2020

Hyperammonemic coma due to Proteus infection

1 reference

12 December 2020

The nutritional management of urea cycle disorders

1 reference

12 December 2020

Ammonia toxicity to the brain: effects on creatine metabolism and transport and protective roles of creatine

1 reference

12 December 2020

Pathophysiology of hepatic encephalopathy: a new look at ammonia

1 reference

12 December 2020

Neurological implications of urea cycle disorders

1 reference

12 December 2020

Therapeutic efficacy of L-ornithine-L-aspartate infusions in patients with cirrhosis and hepatic encephalopathy: results of a placebo-controlled, double-blind study.

1 reference

12 December 2020

Interaction of oxidative stress, astrocyte swelling and cerebral ammonia toxicity

1 reference

12 December 2020

The clinical, biochemical, and molecular spectrum of ornithine transcarbamylase deficiency

1 reference

12 December 2020

Brain imaging in urea cycle disorders

1 reference

12 December 2020

Rapid and reliable measurement of highly elevated blood ammonia concentrations in children

1 reference

12 December 2020

Problems in the management of urea cycle disorders

1 reference

12 December 2020

Extracorporeal dialysis in neonatal hyperammonemia: modalities and prognostic indicators.

1 reference

12 December 2020

Current concepts in the pathogenesis of urea cycle disorders

1 reference

12 December 2020

Role of glutamine in cerebral nitrogen metabolism and ammonia neurotoxicity

1 reference

12 December 2020

Alternative pathway therapy for urea cycle disorders: twenty years later

1 reference

12 December 2020

Urea cycle defects: management and outcome

1 reference

12 December 2020

Urea metabolism in man.

1 reference

12 December 2020

Measurement of ammonia in human breath with a liquid-film conductivity sensor

1 reference

12 December 2020

Lactulose: a review of its therapeutic and pharmacological properties with particular reference to ammonia metabolism and its mode of action of portal systemic encephalopathy

1 reference

12 December 2020

Citrin and aralar1 are Ca(2+)-stimulated aspartate/glutamate transporters in mitochondria

1 reference

12 December 2020

Mechanisms of hyperammonemia

1 reference

12 December 2020

Amino Acid Metabolism in Man

1 reference

12 December 2020

Ammonium in nervous tissue: transport across cell membranes, fluxes from neurons to glial cells, and role in signalling

1 reference

12 December 2020

Intestinal mucosal amino acid catabolism

1 reference

12 December 2020

Liver glutamine metabolism

1 reference

12 December 2020

Comparison of lactulose and neomycin in the treatment of chronic portal-systemic encephalopathy. A double blind controlled trial

1 reference

12 December 2020

An update on the role of brain glutamine synthesis and its relation to cell-specific energy metabolism in the hyperammonemic brain: further studies using NMR spectroscopy

1 reference

12 December 2020

Effects of hyperammonaemia on brain function

1 reference

12 December 2020

10.1007/S00431-010-1369-2

Identifiers

DOI

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23 August 2017

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PubMed ID

21165747