Neonatal diabetes mellitus (also termed congenital diabetes or diabetes of infancy) is highly likely to be due to an underlying monogenic defect when it occurs under 6 months of age. Early recognition and urgent genetic testing are important for predicting the clinical course and raising awareness of possible additional features, and in many cases these are essential for guiding appropriate and cost-effective treatment. Additionally, early treatment of sulfonylurea-responsive types of neonatal diabetes may improve neurological outcomes. It is important to distinguish neonatal diabetes mellitus from other causes of hyperglycemia in the newborn. Other causes include infection, stress, and inadequate pancreatic insulin production in the preterm infant, among others. Insulin-dependent hyperglycemia that persists longer than a week should raise suspicion for neonatal diabetes mellitus and prompt genetic testing.
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