Hepatic Medicine: Evidence and Research

Publisher: Dove Medical Press

E-ISSN: 1179-1535|2018|default|105-115

ISSN: 1179-1535

Source: Hepatic Medicine: Evidence and Research, Vol.2018, Iss.default, 2018-09, pp. : 105-115

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Abstract

Acute hyperammonemia may induce a neurological impairment leading to an acute life threatening condition. Coma duration, ammonia peak level and hyperammonemia duration are the main risk factors of hyperammonemia related neurological 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 needs to be addressed carefully. The management of hyperammonemia includes: (1) identification of precipitating factors and cerebral edema presence, (2) 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 extra-corporeal therapies. In case of severe coma, transcranial Doppler ultrasound could be the method of choice to non-invasively monitor cerebral blood flow and titrate therapies.