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Urea cycle disorders, Arginine Chloride in the treatment of hyperammonemia


Authors: S. Mečířová
Authors‘ workplace: Klinika pediatrie a dědičných poruch metabolismu (KPDPM) 1. LF UK a VFN v Praze
Published in: Klin. Biochem. Metab., 29, 2021, No. 4, p. 209-212

Overview

The text presents a short overview of clinical manifestation, diagnostics and therapy of urea cycle disorders, emphasises the role of Arginin chloride in the therapy of hyperammonemia. Two case reports of one of the most frequent type of urea cycle disorders, ornithin transcarbamylase deficiency, are presented at the end.

Keywords:

Urea cycle disorders – OTCD – ornithin transcarbamylase deficiency – arginin chloride


Sources
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  4. Brusilow, S. W. Arginine, an indispensable amino acid for patients with inborn errors of urea synthesis. J Clin. Invest., 1984, 74, č. 6, s. 2144-2148. doi: 10.1172/JCI111640. PMID: 6511918; PMCID: PMC425406.
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