neonatal diabetes – all you need to know

Neonatal diabetes is type of diabetes mellitus that affect the neonates that is, young infants below 6 months of age. Here, the ability to produce insulin is disturbed and so glucose accumulate in the body. It is controlled by single gene (monogenic). This very rare disease occur one in five lakhs births. It is different from type 1 diabetes as type 1 diabetes does not affect children below 6 months of age.

Types of neonatal diabetes

There are two different type of neonatal diabetes:

neonatal diabetes
a child with neonatal diabetes
  1. Permanent neonatal diabetes (PNDM)

It does not resolve and will continue to last long throughout the life.

It occurs due to mutation in KCNJ11 gene and ABCC8 gene.

 

  1. Transient neonatal diabetes (TNDM)

It resolves within few months that is within 3 weeks.

It occurs due to overexpression of two gene candidates that is, ZAC (a zinc finger protein) and HYMAI (hydatiform mole-associated and imprinted transcript) at the chromosome region 6q24. This overexpression occurs due to

  • Paternal UPD6 (uniparental isodisomy)
  • TNDM1 duplication
  • Hypo methylation of TNDM1 region

 

Clinical features of neonatal diabetes

  • Thirst and frequent urination
  • Ketoacidosis
  • Dehydration
  • Intra uterine growth restriction
  • Hyperglycemia
  • Hypoglycemia

Refer: signs and symptoms of diabetes

Diagnosis

Molecular analysis and gene testing is to be done to differentially diagnose neonatal diabetes into PNDM and TNDM.

Management

A constant follow up and therapeutic plan should be adopted. Use of insulin pumps can be helpful. It can be treated with oral sulfonyl urea like glyburide

Differentiating type 1 and type 2 diabetes

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