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Neonatal Diabetes

  • Mark A. Sperling

Abstract

Neonatal Diabetes Mellitus (NDM) is defined as persistent hyperglycemia occurring in the first months of life, lasting for more than two weeks and requiring insulin for management1. It is considered distinct from autoimmune type 1 DM which manifests after the first three to six months of life2. Whereas type 1 DM in the first year of life is characterized by markers of autoimmunity such as ICA, GAD65, or IA2 antibodies, neonatal DM does not share these characteristics. Rather, NDM is increasingly recognized as a genetic disorder with disturbances in the genes regulating islet organogenesis and function, including insulin secretion3,4. The tools of molecular biology have permitted remarkable progress in delineating the defects responsible for transient and permanent NDM, providing a rational basis for classification, prognosis and management3,4. Moreover, the identified defects in genes during islet organogenesis or insulin secretion are being investigated as potential contributors to diabetes mellitus in later life3-5. Thus, although NDM is rare, with an incidence of approximately 1:500,000 births1 , it has been quite instructive for providing insight on the regulation of insulin secretion and action3,4.

Keywords

Insulin Secretion Neonatal Diabetes Uniparental Disomy Neonatal Diabetes Mellitus 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 2003

Authors and Affiliations

  • Mark A. Sperling
    • 1
  1. 1.Children’s Hospital of PittsburghUniversity of Pittsburgh School of MedicineUSA

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