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Neonatal Isovaleric Acidemia Associated with Hyperammonemia

  • M. Yoshino
  • I. Yoshida
  • F. Yamashita
  • M. Mori
  • C. Uchiyama
  • M. Tatibana
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 153)

Abstract

Isovaleric acidemia (IVA) is an inborn error of leucine metabolism due to a congenital deficiency of isovaleryl-CoA dehydrogenase (Rhead and Tanaka, 1980). Association of significant hyperammonemia with the disease has not been previously described. The present paper reports on a neonate with IVA who developed hyperammonemia. This observation may indicate that secondary derangement of ammonia metabolism can take place in IVA and that IVA should be included in the differential diagnosis of hyperammonemia in the neonatal period.

Keywords

Orotic Acid Calcium Gluconate Maple Syrup Urine Disease Body Odour Isovaleric Acid 
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

  • M. Yoshino
    • 1
  • I. Yoshida
    • 1
  • F. Yamashita
    • 1
  • M. Mori
    • 2
  • C. Uchiyama
    • 2
  • M. Tatibana
    • 2
  1. 1.Department of PediatricsKurume UniversityKurumeJapan
  2. 2.Department of BiochemistryChiba UniversityChibaJapan

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