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Acute pediatric hyperammonemia: current diagnosis and management strategies.

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.

SUBMITTER: Savy N

PROVIDER: S-EPMC6140721 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature

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Acute pediatric hyperammonemia: current diagnosis and management strategies.

Savy Nadia N Brossier David D Brunel-Guitton Catherine C Ducharme-Crevier Laurence L Du Pont-Thibodeau Geneviève G Jouvet Philippe P

Hepatic medicine : evidence and research 20180912

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 ...[more]

PMID: 30254497

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Dataset Information

Acute pediatric hyperammonemia: current diagnosis and management strategies.

Publications

Acute pediatric hyperammonemia: current diagnosis and management strategies.

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