Neonatal Diabetes Mellitus: An Update on Diagnosis and Management

Clin Perinatol. 2018 Mar;45(1):41-59. doi: 10.1016/j.clp.2017.10.006. Epub 2017 Dec 16.


Neonatal diabetes mellitus is likely to be due to an underlying monogenic defect when it occurs at less than 6 months of age. Early recognition and urgent genetic testing are important for predicting the clinical course and raising awareness of possible additional features. Early treatment of sulfonylurea-responsive types of neonatal diabetes may improve neurologic outcomes. It is important to distinguish neonatal diabetes mellitus from other causes of hyperglycemia in newborns. Other causes include infection, stress, inadequate pancreatic insulin production in preterm infants, among others. This review explores the diagnostic approach, mutation types, management, and clinical course of neonatal diabetes.

Keywords: Genetic; Glyburide; Insulin; Monogenic diabetes; Neonatal diabetes.

Publication types

  • Review

MeSH terms

  • Diabetes Mellitus / congenital*
  • Diabetes Mellitus / diagnosis
  • Diabetes Mellitus / drug therapy
  • Diabetes Mellitus / genetics
  • Humans
  • Hyperglycemia / congenital
  • Hypoglycemic Agents / therapeutic use
  • Infant, Extremely Premature
  • Infant, Newborn
  • Infant, Premature
  • Insulin / therapeutic use
  • Mutation
  • Sulfonylurea Compounds / therapeutic use


  • Hypoglycemic Agents
  • Insulin
  • Sulfonylurea Compounds