Abstract

NOGALES, Andrea et al. Hyperammonaemia in children: Classification and therapeutic options. Gen [online]. 2013, vol.67, n.1, pp.11-15. ISSN 0016-3503.

Introduction: Hyperammonaemia occurs secondarily by increased production of ammonia, as gastrointestinal bleeding or decreased elimination, as occurs in inborn errors of metabolism, especially in those with defects in the urea cycle, liver failure or drugs. Objective: To classify the report hyperammonaemia and therapeutic options in children, its clinical approach and review of the literature. Methods: Prospective, descriptive and transversal children with hyperammonaemia. Variables: age, gender, etiology, ammonia levels, clinical treatment. Results: 21 patients, 12 (57,12%) males and 9 (42,88%) females. Mean age 3,91 years (range: <1m-14a). ammonium 214,66 mmol / l (range :110-980), classified according to severity: no hepatic impairment 11/21 with 99,44 average ammonium and 201 mmol / l in Hyperammoanemia mild and moderate respectively. Clinical and laboratory liver failure 10/21 with ammonium averaging 114,44, 287,51 and 756,66 as mild, moderate and severe hyperammonemia, with a significant difference between the level of ammonia and the presence or absence of liver failure (p < 0,0001), 5/10 with liver failure admitted to intensive care, 4 of them had hepatic encephalopathy, a patient died. Etiology: An inborn error of metabolism 33,33%, 23,80% drug toxicity, fulminant viral hepatitis and other viruses 19,04% 9,52% 4,76% autoimmune hepatitis and urosepsis 4,76%. In mild-moderate cases were given oral lactulose Dose 19/21 and by enema rectal 7/21 with L-carnitine in 15/21 and further severe Hyperammonemia sodium benzoate 4/21 and was indication of hemodialysis in 3 patients. Protein restriction at all, vitamin therapy and 6 children treated with ácidoursodeoxicólico. Conclusions: hyperammonemia is multifactorial, requires early diagnosis, classification of severity allows early treatment to avoid complications and development of irreversible neurological sequelae.

Keywords : Hyperammonemia; liver failure; hepatic encephalopathy; sodium benzoate; lactulose; L-carnitine.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )