ReviewHepatic medicine : evidence and research2018
Acute pediatric hyperammonemia: current diagnosis and management strategies.
Review in Hepatic medicine : evidence and research, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers.
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Who cites it
43 citing papers in PubMed.
- Real world experience of carglumic acid for methylmalonic and propionic acidurias: the second interim analysis of the multicentre observational PROTECT study.Orphanet journal of rare diseases · 2026Observational
- "Why won't the ammonia go down?": ammonia management while on continuous kidney replacement therapy.Pediatric nephrology (Berlin, Germany) · 2026Article
- Perioperative management of pediatric patients with inborn errors of metabolism during liver transplantation.World journal of transplantation · 2026Review
- A Complex Case of Ornithine Transcarbamylase Deficiency in a Patient With Severe Comorbid Conditions.Clinical case reports · 2026Article
- Hypoaminoacidemia and Pyroglutamic Aciduria: Potential Biomarkers in Malnutrition-Related Hyperammonemia.JIMD reports · 2026Article
- The circulatory arrest recovery ammonia problem (CARAP) hypothesis: A proton magnetic resonance spectroscopy (The Journal of thoracic and cardiovascular surgery · 2025Article
- A Machine Learning Approach to Differentiate Congenital and Transient Neonatal Hyperammonemia: A 10-Year Case Series.Cureus · 2025Article
- Early continuous blood purification and timely liver transplantation in a neonatal-onset ornithine transcarbamylase deficiency: a case report.Translational pediatrics · 2025Article
- Practical Considerations for the Diagnosis and Management of Isovaleryl-CoA-Dehydrogenase Deficiency (Isovaleric Acidemia): Systematic Search and Review and Expert Opinions.International journal of neonatal screening · 2025Review
- [Interpretation of the "Expert consensus on the diagnosis and treatment of neonatal hyperammonemia"].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2025Article
- Chemotactic Zn micromotor for treatment of high blood ammonia-associated hepatic encephalopathy.Nature communications · 2025Article
- Hyperammonemic encephalopathy requiring hemodialysis in a child with distal renal tubular acidosis.Journal of nephrology · 2025Article
- Perspectives on long-term medical management of urea cycle disorders: insights from a survey of UK healthcare professionals.Orphanet journal of rare diseases · 2025Article
- Global research dynamics in urea cycle disorders: a bibliometric study highlighting key players and future directions.Orphanet journal of rare diseases · 2025Article
- Imaging features, classification and clinical features of intrahepatic congenital portosystemic shunts.Journal of ultrasound · 2025Article
- Emergency Management of Intoxication-Type Inherited Metabolic Disorders.Journal of inherited metabolic disease · 2025Review
- Perioperative care in pediatric OTC deficiency and goldenhar syndrome: a case report.Frontiers in pediatrics · 2025Article
- Engineered Probiotics for the Management of Congenital Metabolic Diseases: A Systematic Review.Preventive nutrition and food science · 2024Review
- Distinct neonatal hyperammonemia and liver synthesis dysfunction: case report of a severe MEGDHEL syndrome.Frontiers in pediatrics · 2024Article
- Hyperosmolarity in children with hyperammonemia: a risk of brain herniation at the start of renal replacement therapy.Frontiers in pediatrics · 2024Article
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Authors and funding
6 authors.
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Abstract
Acute hyperammonemia may induce a neurologic impairment leading to an acute life-threatening condition. Coma duration, ammonia peak level, and hyperammonemia duration are the main risk factors of hyperammonemia-related neurologic deficits and death. In children, hyperammonemia is mainly caused by severe liver failure and inborn errors of metabolism. In an acute setting, obtaining reliable plasma ammonia levels can be challenging because of the preanalytical difficulties that need to be addressed carefully. The management of hyperammonemia includes 1) identification of precipitating factors and cerebral edema presence, 2) a decrease in ammonia production by reducing protein intake and reversing catabolism, and 3) ammonia removal with pharmacologic treatment and, in the most severe cases, with extracorporeal therapies. In case of severe coma, transcranial Doppler ultrasound could be the method of choice to noninvasively monitor cerebral blood flow and titrate therapies.
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