Author/Editor		Žerjav-Tanšek, Mojca; Bratanič, Nevenka; Battelino, Tadej
Title		Diagnoza in zdravljenje akutne hiperamoniemije
Translated title		Diagnosis and early management of acute hyperammonaemia
Type		članek
Source		Zdrav Vestn
Vol. and No.		Letnik 79, št. 2
Publication year		2010
Volume		str. 164-72
Language		slo
Abstract		Acute hyperammonaemia is a consequence of inborn defects of urea cycle or acute liver damage. Clinical presentation varies considerably with the age of the aff ected individual and includes nausea, vomiting, neuropsychiatric symptoms, convulsions and progressive lethargy with a deepening coma. Ammonia concentration above 200 microM is a medical emergency and requires prompt treatment, regardless of its aetiology. The acute management includes restriction of protein intake, glucose infusion suffi cient to maintain anabolic state, administration of ammonia scavengers (sodium benzoate, sodium phenylacetate, sodium phenylbutyrate), administration of urea cycle substrates and haemodialysis when the concentration of ammonia is exceedingly high or does not respond promptly to the medical treatment. Elevated ammonia concentration is neurotoxic and causes irreversible glial and neuronal damage resulting in cerebral oedema with poor outcome. Early diagnosis and prompt treatment are crucial and may considerably improve the clinical course.
Descriptors		METABOLIC DISEASES AMMONIA EMERGENCIES PHENYLACETATES