Diabetes Mellitus, Permanent Neonatal, 3

Alternative Names

  • PNDM3
  • Developmental Delay, Epilepsy, and Neonatal Diabetes 2
  • DEND2
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WHO-ICD-10 version:2010

Certain conditions originating in the perinatal period

OMIM Number


Mode of Inheritance

Autosomal dominant -Autosomal recessive

Gene Map Locus



Permanent neonatal diabetes mellitus-3 (PNDM3) is characterized by the onset of mild to severe hyperglycemia within the first months of life, and requires lifelong therapy . Some patients also have neurologic features, including developmental delay and epilepsy. The triad of developmental delay, epilepsy, and neonatal diabetes is known as DEND. [From OMIM]

Epidemiology in the Arab World

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Other Reports

United Arab Emirates

Deeb et al. (2016) examined Neonatal Diabetes Mellitus (NDM) patients and characterized their genetic and clinical features. The authors found 25 cases of NDM in Abu Dhabi between the years 1985-2013, giving an incidence rate of 1:29,241 live births. Of these, 23 were Permanent Neonatal Diabetes Mellitus (PNDM) cases and 2 patients had Transient Neonatal Diabetes Mellitus (TNDM). Genetic analysis of this cohort identified a heterozygous H410Y ABCC8 variant in a 15-year-old male patient. The affected individual was born full term with a birth weight of 2.4 kg to a non-consanguineous family. He was diagnosed with NDM at 25 weeks. At 15 years of age he had normal growth and intelligence and was on insulin pump therapy of 1.3U/kg/day. His 45-year-old mother was a carrier for the variant and had glucose intolerance with HbA1c of 5.9% on diet control.

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