Acute pediatric hyperammonemia: current diagnosis and management strategies

Nadia Savy,1 David Brossier,2 Catherine Brunel-Guitton,1 Laurence Ducharme-Crevier,1 Geneviève Du Pont-Thibodeau,1 Philippe Jouvet1 1Department of Pediatrics, CHU Sainte-Justine, University of Montreal, Montreal, QC, Canada; 2Department of Pediatrics, Pediatric Intensive Care Unit, CHU Ca...

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Autores principales:	Savy N, Brossier D, Brunel-Guitton C, Ducharme-Crevier L, Du Pont-Thibodeau G, Jouvet P
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2018
Materias:	Pediatrics Critical Care hyperammonemia sodium phenylacetate sodium benzoate hemodialysis. Diseases of the digestive system. Gastroenterology RC799-869
Acceso en línea:	https://doaj.org/article/8f51fa196350445096f62770cdd57065
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spelling	oai:doaj.org-article:8f51fa196350445096f62770cdd570652021-12-02T06:59:54ZAcute pediatric hyperammonemia: current diagnosis and management strategies1179-1535https://doaj.org/article/8f51fa196350445096f62770cdd570652018-09-01T00:00:00Zhttps://www.dovepress.com/acute-pediatric-hyperammonemia-current-diagnosis-and-management-strate-peer-reviewed-article-HMERhttps://doaj.org/toc/1179-1535Nadia Savy,1 David Brossier,2 Catherine Brunel-Guitton,1 Laurence Ducharme-Crevier,1 Geneviève Du Pont-Thibodeau,1 Philippe Jouvet1 1Department of Pediatrics, CHU Sainte-Justine, University of Montreal, Montreal, QC, Canada; 2Department of Pediatrics, Pediatric Intensive Care Unit, CHU Caen, Caen, France 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. Keywords: pediatrics, critical care, hyperammonemia, sodium phenylacetate, sodium benzoate, hemodialysisSavy NBrossier DBrunel-Guitton CDucharme-Crevier LDu Pont-Thibodeau GJouvet PDove Medical PressarticlePediatricsCritical Carehyperammonemiasodium phenylacetatesodium benzoatehemodialysis.Diseases of the digestive system. GastroenterologyRC799-869ENHepatic Medicine: Evidence and Research, Vol Volume 10, Pp 105-115 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	Pediatrics Critical Care hyperammonemia sodium phenylacetate sodium benzoate hemodialysis. Diseases of the digestive system. Gastroenterology RC799-869
spellingShingle	Pediatrics Critical Care hyperammonemia sodium phenylacetate sodium benzoate hemodialysis. Diseases of the digestive system. Gastroenterology RC799-869 Savy N Brossier D Brunel-Guitton C Ducharme-Crevier L Du Pont-Thibodeau G Jouvet P Acute pediatric hyperammonemia: current diagnosis and management strategies
description	Nadia Savy,1 David Brossier,2 Catherine Brunel-Guitton,1 Laurence Ducharme-Crevier,1 Geneviève Du Pont-Thibodeau,1 Philippe Jouvet1 1Department of Pediatrics, CHU Sainte-Justine, University of Montreal, Montreal, QC, Canada; 2Department of Pediatrics, Pediatric Intensive Care Unit, CHU Caen, Caen, France 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. Keywords: pediatrics, critical care, hyperammonemia, sodium phenylacetate, sodium benzoate, hemodialysis
format	article
author	Savy N Brossier D Brunel-Guitton C Ducharme-Crevier L Du Pont-Thibodeau G Jouvet P
author_facet	Savy N Brossier D Brunel-Guitton C Ducharme-Crevier L Du Pont-Thibodeau G Jouvet P
author_sort	Savy N
title	Acute pediatric hyperammonemia: current diagnosis and management strategies
title_short	Acute pediatric hyperammonemia: current diagnosis and management strategies
title_full	Acute pediatric hyperammonemia: current diagnosis and management strategies
title_fullStr	Acute pediatric hyperammonemia: current diagnosis and management strategies
title_full_unstemmed	Acute pediatric hyperammonemia: current diagnosis and management strategies
title_sort	acute pediatric hyperammonemia: current diagnosis and management strategies
publisher	Dove Medical Press
publishDate	2018
url	https://doaj.org/article/8f51fa196350445096f62770cdd57065
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Acute pediatric hyperammonemia: current diagnosis and management strategies

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