Permanent neonatal diabetes mellitus

Common Name(s)

Permanent neonatal diabetes mellitus

Permanent neonatal diabetes mellitus (PNDB) is a type of diabetes that appears within the first 6 months of life and persists throughout life. Affected individuals have slow growth before birth followed by hyperglycemia, dehydration and failure to thrive in infancy. Some individuals also have neurological problems including developmental delay and epilepsy; when these problems are present with PNDB, it is called DEND syndrome. A few individuals with PNDB also have an underdeveloped pancreas and may have digestive problems. PNDB is caused by mutations in any one of several genes (some of which have not yet been identified) including the KCNJ11, ABCC8, and INS genes. It may be inherited in an autosomal recessive or autosomal dominant manner. Treatment includes rehydration, insulin therapy and/or long-term therapy with oral sulfonylureas (in some cases).
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Permanent neonatal diabetes mellitus" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Permanent neonatal diabetes mellitus" returned 10 free, full-text research articles on human participants. First 3 results:

Permanent neonatal diabetes mellitus: prevalence and genetic diagnosis in the SEARCH for Diabetes in Youth Study.
 

Author(s): Roopa Kanakatti Shankar, Catherine Pihoker, Lawrence M Dolan, Debra Standiford, Angela Badaru, Dana Dabelea, Beatriz Rodriguez, Mary Helen Black, Giuseppina Imperatore, Andrew Hattersley, Sian Ellard, Lisa K Gilliam,

Journal: Pediatr Diabetes. 2013 May;14(3):174-80.

 

Neonatal diabetes mellitus (NDM) is defined as diabetes with onset before 6 months of age. Nearly half of individuals with NDM are affected by permanent neonatal diabetes mellitus (PNDM). Mutations in KATP channel genes (KCNJ11, ABCC8) and the insulin gene (INS) are the most common causes of PNDM.

Last Updated: 23 Apr 2013

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Three cases of permanent neonatal diabetes mellitus: genotypes and management outcome.
 

Author(s): Hooi Leng Ooi, Loo Ling Wu

Journal: Singapore Med J. 2012 Jul;53(7):e142-4.

 

Neonatal diabetes mellitus (DM) is defined as insulin-requiring DM in the first six months of life. Unlike type 1 DM, it is a monogenic disorder resulting from a de novo mutation in the genes involved in the development of the pancreas, β-cell mass or secretory function. The majority ...

Last Updated: 20 Jul 2012

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Permanent neonatal diabetes mellitus: same mutation, different glycemic control with sulfonylurea therapy on long-term follow-up.
 

Author(s): Banu Kücükemre Aydin, Rüveyde Bundak, Firdevs Baş, Hülya Maraş, Nurçin Saka, Hülya Günöz, Feyza Darendeliler

Journal: J Clin Res Pediatr Endocrinol. 2012 Jun;4(2):107-10.

 

Permanent neonatal diabetes mellitus (PNDM) is a rare condition presenting before six months of age. Mutations in the genes encoding the ATP-sensitive potassium (KATP) channel are the most common causes. Sulfonylurea (SU) therapy leads to dramatic improvement in diabetes control and ...

Last Updated: 7 Jun 2012

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Reviews from the PubMed Database

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The terms "Permanent neonatal diabetes mellitus" returned 0 free, full-text review articles on human participants.

 
 
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