Abstract
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.
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© 1982 Springer Science+Business Media New York
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Saudubray, J.M., Coudé, F.X., Ogier, H., Cathelineau, L., Briand, P., Charpentier, C. (1982). Hyperammonemia Secondary to Hereditary Organic Acidurias : A Study of 29 Cases. In: Lowenthal, A., Mori, A., Marescau, B. (eds) Urea Cycle Diseases. Advances in Experimental Medicine and Biology, vol 153. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-6903-6_18
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DOI: https://doi.org/10.1007/978-1-4757-6903-6_18
Publisher Name: Springer, Boston, MA
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