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Nonketotic Hyperglycinemia

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Inborn Metabolic Diseases

Abstract

Hyperglycinemia represents a group of disorders characterized by elevated concentrations of glycine in body fluids. Two types exist, the nonketotic and the ketotic type [1]. Nonketotic hyperglycinemia (NKH) is a disorder of glycine degradation due to a primary defect in the glycine cleavage system (GCS). NKH is a relatively frequent metabolic cause of overwhelming illness in infancy. In the ketotic type. the most striking feature is ketoacidosis, which begins early in life and in which hyperglycinemia is secondarily associated with organic academia [1].

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Authors
  1. K. Tada
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Editor information

Editors and Affiliations

  1. University Children’s Hospital, Oostersingel 59, 9713 EZ, Groningen, The Netherlands

    John Fernandes M.D.

  2. Hospital Necker-Enfants Malades, 149, Rue de Sèvres, 75743, Paris Cedex, France

    Jean-Marie Saudubray M.D.

  3. International Institute of Cellular and Molecular Pathology, Avenue Hippocrate 75, 1200, Brussels, Belgium

    Georges Van den Berghe M.D.

  4. Sendai, Japan

    K. Tada

  5. Portland, Oregon, USA

    N. R. M. Buist

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© 1995 Springer-Verlag Berlin Heidelberg

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Tada, K. (1995). Nonketotic Hyperglycinemia. In: Fernandes, J., Saudubray, JM., Van den Berghe, G., Tada, K., Buist, N.R.M. (eds) Inborn Metabolic Diseases. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-03147-6_17

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  • DOI: https://doi.org/10.1007/978-3-662-03147-6_17

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-03149-0

  • Online ISBN: 978-3-662-03147-6

  • eBook Packages: Springer Book Archive

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