Neonatal diabetes mellitus, often called hyperglycemia, occurs within the first month of birth ad lasts for a minimum of three weeks requiring insulin therapy. Diabetes in newborns is a very rare occurrence and is not autoimmune, with one in every 100,000 to 500,000 babies being diagnosed. There are three names used for diabetes in newborns: neonatal diabetes mellitus (NDM), transient neonatal diabetes mellitus (TNDM), and permanent neonatal diabetes mellitus (PNDM). There is no way to distinguish between the different forms of diabetes in newborns without expensive genetic testing. Most treatment is dependent on the appearance of symptoms.
Diabetes in Newborns – TNDM
TNDM is defined as hyperglycemia occurring in the first months of life and lasting at least two weeks and requiring insulin therapy. Most cases of TNDM resolve themselves spontaneously within 4 months with case studies showing short periods of insulin dependence before the disease clears and a high probability of remission in childhood or adulthood. The prevalence of TNDM is 1 in every 45,000 to 60,000 live births. The hypothesized etiology is a delay in the maturation of camp mediated insulin release. Clinical features of TNDM are intrauterine growth restriction, proneness for birth asphyxia, dehydration, emaciation, polyuria and poydipsea with prevalence for septicemia and urinary tract infections.
Another form of TNDM, 6q24-related transient neonatal diabetes mellitus (6q24-TNDM) is defined as a form of transient neonatal diabetes caused by genetic abnormalities of the imprinted locus at 6q24. The clinical features are intrauterine growth restriction, hyperglycemia in the neonatal period that resolves by 18 months, dehydration, and an absence of ketoacidosis. Macroglosia and umbilical hernia are also signs. Full-blown diabetes mellitus begins at one week and can last from three months to a year with the need for insulin gradually declining. Diabetes may come back in childhood or adolescence or during pregnancy in women. Primary treatment of 6q24-TDM is hydration and insulin until blood glucose stabilizes.
Diabetes in Newborns – PNDM
Of those with TNDM thirty percent of newborns are likely to develop PNDM. Permanent diabetes mellitus is uncommon in babies and is usually due to pancreatic dysgenesis. Babies with a mutation in KCNJ11 or ABCC8 with PNDM can be treated with oral sulphonylureas rather than insulin. 20% of cases of PNDM have a KCNJ11 mutation, with associated neurological findings called END syndrome (development delay, epilepsy, and neonatal diabetes mellitus). Babies with affected IPF-1 or GCK will require insulin (neither rapid-acting nor short-acting) as there is no beta-cell activity. Newborns with PNDM should also be given a higher caloric intake for weight gain Babies with PNDM are likely to develop the vascular complications of diabetes at an earlier rate.
