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>Journals >Acta Pediátrica de México >Year 2005, Issue 6


Arnold-Almaraz K, Olivares-Sandoval Z, Revilla-Estivill NF, Ibarra-González I, Belmont-Martínez L, Vela-Amieva M
Management of hyperamonemia in children
Acta Pediatr Mex 2005; 26 (6)

Language: Español
References: 31
Page: 313-324
PDF: 104.71 Kb.


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ABSTRACT

Hyperammonemia is the chronic or acute increase of seric ammonium. It is associated with a high morbi-mortality. Hyperammonemia is a very frequent biochemical problem in patients with inborn metabolic diseases, especially in those with urea cycle disorders, disorders of leucine catabolism, and propionic or methylmalonic acidemia. It can also be present in several non congenital dysfunctions. It should be treated and corrected immediately, lest it causes severe and irreversible neurological damage leading to intellectual and motor disability. The management of these patients requires a combination of medical and dietetic treatment. The priority is to normalize the ammonium concentrations as quickly as possible,. The treatment has two phases: acute and chronic. The chronic treatment is usually long term and in many occasions, for life. Basically it requires on nutritional recommendations. The objective of this review is to offer the pediatrician a current guide for the treatment of patients with congenital hyperammonemia.


Key words: Ammonium, hyperammonemia, inborn errors of metabolism, antiammonium treatment, intellectual invalidity, motor invalidity.


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