Hyperammonemia Secondary to Hereditary Organic Acidurias : A Study of 29 Cases

  • J. M. Saudubray
  • F. X. Coudé
  • H. Ogier
  • L. Cathelineau
  • P. Briand
  • C. Charpentier
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 153)

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.

Keywords

Blood Ammonia Organic Aciduria Propionic Acidemia Carbamyl Phosphate Methylmalonic Acidemia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 1982

Authors and Affiliations

  • J. M. Saudubray
    • 1
  • F. X. Coudé
    • 1
  • H. Ogier
    • 1
  • L. Cathelineau
    • 1
  • P. Briand
    • 1
  • C. Charpentier
    • 1
  1. 1.INSERM U 12, Laboratoire de Biochimie GénétiqueHôpital NeckerParis Cedex 15France

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