Transient neonatal diabetes mellitus

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Disease Overview

Transient neonatal diabetes mellitus (TNDB) is a type of diabetes that appears within the first few weeks of life but is transient; affected infants go into remission within a few months, with possible relapse to permanent diabetes in adolescence or adulthood.[5409] Affected individuals have slow growth before birth followed by hyperglycemia, dehydration and failure to thrive in infancy.[5406] Approximately 70% of cases are caused by the overactivity of certain genes in a region of the long (q) arm of chromosome 6 called 6q24.[5410][5414] These cases are referred to as 6q24-related TNDB; most (but not all) of these cases are not inherited.[5414] Other genetic causes include mutations in the KCNJ11 and ABCC8 genes, which usually cause permanent neonatal diabetes.[5410] Treatment may include rehydration and intravenous insulin at the time of diagnosis, followed by subcutaneous insulin.[5410]


Synonyms

  • TNDM1
  • TNDM
  • DMTN
  • Diabetes mellitus, transient neonatal
  • Chromosome 6-associated transient diabetes mellitus
  • Diabetes mellitus, 6q24-related transient neonatal

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National Organization for Rare Disorders