Transient Neonatal Diabetes Mellitus

Information about:

Transient Neonatal Diabetes Mellitus

(Transient neonatal diabetes mellitus 1, TNDM1, OMIM #601410)

Transient neonatal diabetes (type 1) (TNDM1) is characterised by diabetes mellitus that develops in the first six weeks of life, and resolves by the age of 18 months (Temple et al, 2012; Mackay et al 2014).  It is caused by over-expression of imprinted genes at chromsome 6q24 (Kamiya et al 2000, Gardner et al, 2000).  TNDM1 is distinct from transient neonatal diabetes caused by mutations of other genes (Polak & Cave 2007; Flanagan et al 2007), including TNDM2 (OMIM #610374) associated with mutation of ABCC8, and TNDM3 (OMIM #610582) caused by mutation of KCNJ11.  Prevalence of TNDM in the UK was initially estimated at 1:400,000 as reported (Shield et al 1997); but more recent calculations estimate total neonatal diabetes incidence of 1:215,000 to 1:400,000 (Polak & Shield 2004, Stanik et al 2007, Wiedemann et al 2010).

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