L-carnitine in Hyperammonemia and Hepatic Encephalopathy

J, Missler; Zwingmann, Claudia

doi:10.5772/29855

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Initial Management Of Hyperammonemia1

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2018

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“…In rats, 1 H- and 13 C-MRS have shown to improve the mitochondrial energy metabolism in the brain and in muscles with l -carnitine. 32 …”

Section: Initial Management Of Hyperammonemiamentioning

confidence: 99%

Acute pediatric hyperammonemia: current diagnosis and management strategies

Savy¹,

Brossier²,

Brunel‐Guitton³

et al. 2018

HMER

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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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“…In rats, 1 H- and 13 C-MRS have shown to improve the mitochondrial energy metabolism in the brain and in muscles with l -carnitine. 32 …”

Section: Initial Management Of Hyperammonemiamentioning

confidence: 99%