STUDY OF THE EFFICIENCY OF THE APPLIED DIETARY MANAGEMENT IN PATIENTS WITH UREA CYCLE DISORDER

Abstract

The urea cycle was first describe in 1932 by Kerbs and Henseleit (Summar et al., 2008). The urea cycle is a series of enzymatic reactions converting highly toxic ammonia into less toxic urea (Testai & Gorelick, 2010). In the liver ammonium nitrogen from dietary protein sources and from the breakdown of endogenous protein is converted into urea. Urea unlike ammonia that is nontoxic, water-soluble, and is easily excreted from the body through the kidney as a component of urine (Deignan et al., 2008). The urea cycle involves a series of biochemical steps in which nitrogen from protein metabolism is converted to urea, deficiency of one of the enzymes involved in this process causes disorders collectively known as urea cycle disorders (Gropman et al., 2007).