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Neonatal insulin-dependent diabetes mellitus

MedGen UID:
Concept ID:
HPO: HP:0000857

Conditions with this feature

Pancreatic beta cell agenesis with neonatal diabetes mellitus
MedGen UID:
Concept ID:
Disease or Syndrome
Pancreatic agenesis 1
MedGen UID:
Concept ID:
Disease or Syndrome
In some cases, people with permanent neonatal diabetes mellitus also have certain neurological problems, including developmental delay and recurrent seizures (epilepsy). This combination of developmental delay, epilepsy, and neonatal diabetes is called DEND syndrome. Intermediate DEND syndrome is a similar combination but with milder developmental delay and without epilepsy.\n\nA small number of individuals with permanent neonatal diabetes mellitus have an underdeveloped pancreas. Because the pancreas produces digestive enzymes as well as secreting insulin and other hormones, affected individuals experience digestive problems such as fatty stools and an inability to absorb fat-soluble vitamins.\n\nIndividuals with permanent neonatal diabetes mellitus experience slow growth before birth (intrauterine growth retardation). Affected infants have hyperglycemia and an excessive loss of fluids (dehydration) and are unable to gain weight and grow at the expected rate (failure to thrive).\n\nPermanent neonatal diabetes mellitus is a type of diabetes that first appears within the first 6 months of life and persists throughout the lifespan. This form of diabetes is characterized by high blood sugar levels (hyperglycemia) resulting from a shortage of the hormone insulin. Insulin controls how much glucose (a type of sugar) is passed from the blood into cells for conversion to energy.

Professional guidelines


Benhalima K, Beunen K, Siegelaar SE, Painter R, Murphy HR, Feig DS, Donovan LE, Polsky S, Buschur E, Levy CJ, Kudva YC, Battelino T, Ringholm L, Mathiesen ER, Mathieu C
Lancet Diabetes Endocrinol 2023 Jul;11(7):490-508. Epub 2023 Jun 5 doi: 10.1016/S2213-8587(23)00116-X. PMID: 37290466
Ben-Skowronek I
Genes (Basel) 2021 Feb 24;12(3) doi: 10.3390/genes12030323. PMID: 33668198Free PMC Article
Alexopoulos AS, Blair R, Peters AL
JAMA 2019 May 14;321(18):1811-1819. doi: 10.1001/jama.2019.4981. PMID: 31087027Free PMC Article

Recent clinical studies


Roberts J, Searle J
Pediatr Pathol Lab Med 1995 May-Jun;15(3):477-83. doi: 10.3109/15513819509026984. PMID: 8597835

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