Laboratory for Molecular Diagnostics
Center for Nephrology and Metabolic Disorders

Transient neonatal diabetes mellitus 2

Transient neonatal diabetes mellitus is an autosomal dominant disorder caused by mutations of the ABCC8 gene. The clinical variation within a family can vary significantly from transient or permanent neonatal diabetes mellitus to a typical type 2 diabetes of adulthood.

Pathogenesis

The disease is cause by an activating mutation of the ABCC8 gene.

Management

The disease can be treated by sulfonyl urea.

Systematic

Neonatal diabetes mellitus
Leprechaunism
Mitchell-Riley syndrome
Neonatal diabetes mellitus with congenital hypothyroidism
Pancreatogenic diabetes
Permanent neonatal diabetes mellitus
Rabson-Mendenhall syndrome
Renal cysts and diabetes (RCAD)
Transient neonatal diabetes mellitus 1
Transient neonatal diabetes mellitus 2
ABCC8
Transient neonatal diabetes mellitus 3
X-linked immunodysregulation, polyendocrinopathy, and enteropathy

References:

1.

Babenko AP et al. (2006) Activating mutations in the ABCC8 gene in neonatal diabetes mellitus.

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2.

OMIM.ORG article

Omim 610374 [^]
Update: May 10, 2019