الفهرس | Only 14 pages are availabe for public view |
Abstract IEMs are relatively common disorders in the Middle East and Arabic populations, mainly because of the high rates of consanguinity in the region (total consanguinity up to 67% and first cousin consanguinity up to 49% in some Arab populations) (Tadmouri et al., 2009). Depending on the pathogenesis and the metabolic pathway affected, classical IEMS are grossly classified into different groups: amino acidemias, organic acidemias (OAs), and fatty acid oxidation defects. OAs are the most frequently occurring IEMs among the various types of diseases (Wajner et al., 2002). Although clinical presentations of OAs involve multiple body systems, OAs predominately affects the central nervous system. If OA is not treated, patients may develop irreversible brain damage, leading to mental retardation |