Hyperammonemia Secondary to Hereditary Organic Acidurias : A Study of 29 Cases
Hyperammonia has been reported in several disorders of branched chain amino acids metabolism including propionic, isovaleric, methylmalonic acidemia and B ketothiolase deficiency 1. Nevertheless the true incidence of hyperammonemia and its variation during the course of these diseases are not yet well known. The purpose of this study was to compare the blood ammonia concentrations and the concomitant serum organic acid accumulation in patients with propionic, isovaleric and methylmalonic acidemia.
KeywordsBlood Ammonia Organic Aciduria Propionic Acidemia Carbamyl Phosphate Methylmalonic Acidemia
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