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Published on: November 20, 2015
Neonatal and very-early-onset diabetes mellitus
1Paediatric Endocrinology and INSERM EMI 0363, Hôpital Necker-Enfants Malades, Paris, France. michel.polak@nck.ap-hop-paris.fr
Insights
Transient (TNDM) and permanent neonatal diabetes mellitus (PNDM) are rare infant conditions. Understanding their mechanisms offers insights into fetal development, islet cell function, and type 2 diabetes predisposition.
Area of Science:
- Endocrinology
- Pediatrics
- Genetics
Background:
- Neonatal diabetes mellitus (NDM) encompasses transient (TNDM) and permanent (PNDM) forms, affecting 1 in 400,000-500,000 live births.
- TNDM presents in early infancy, resolves, but often relapses later as type 2 diabetes, suggesting persistent pancreatic dysfunction.
- PNDM involves early-onset insulin secretory failure with identified molecular causes.
Purpose of the Study:
- To explore the underlying mechanisms of TNDM and PNDM.
- To investigate the implications of TNDM for fetal pancreatic development, islet cell physiology, and type 2 diabetes risk.
- To review management strategies for neonatal diabetes, including insulin pump therapy.
Main Methods:
- Literature review and synthesis of existing research on TNDM and PNDM.
- Analysis of clinical presentations and suspected etiologies.
- Discussion of therapeutic approaches.
Main Results:
- TNDM involves temporary diabetes with potential for later relapse, linked to metabolic stress.
- PNDM has diverse molecular underpinnings.
- Insulin pump therapy is a viable option for neonatal insulin management.
Conclusions:
- NDM pathogenesis offers insights into broader diabetes development and physiology.
- Further research into TNDM mechanisms may illuminate type 2 diabetes predisposition.
- Effective insulin management is crucial for infants with PNDM.
Abstract:
Transient (TNDM) and permanent neonatal diabetes mellitus (PNDM) are rare conditions occurring in one in 400,000-500,000 live births. In TNDM, growth-retarded infants develop diabetes in the first few weeks of life only to go into remission in a few months with later relapse as permanent type 2 diabetes, often around the time of adolescence. We believe that pancreatic dysfunction in this condition is maintained throughout life with relapse initiated at times of metabolic stress such as puberty or pregnancy. The mechanisms involved in this rare condition may inform on fetal pancreatic development, islet cell physiology and predisposition to type 2 diabetes. In PNDM, insulin secretory failure occurs in the early postnatal period. A number of conditions are associated with PNDM, some of which have been elucidated at the molecular level. Insulin therapy is difficult to manage in the neonatal period, and in experienced hands, the insulin pump may provide a valuable tool to administer insulin.
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