Abstract
The rooted conviction according to which the childhood diabetes should only be an autoimmune diabetes is gradually disappearing, thanks to the discovery of not autoimmune paediatric diabetes.
Many of these forms of diabetes are a direct consequence of the mutation of a single gene, involved into the insulin secretion mechanism of the pancreatic beta cells. Mutations in genes directly involved in insulin secretion or that limit the ability of the pancreas to produce insulin are the cause of the major non-autoimmune diabetes forms of the paediatric age (monogenic diabetes). The monogenic diabetes accounts for 1–2 % of all cases of diabetes in the paediatric-adolescent age. The apparent increasing in its prevalence is probably due to the improvement of the diagnostic methods. Nowadays there have been described 13 forms of MODY, the mitochondrial diabetes, the secondary diabetes and the syndromic diabetes.
In this chapter, all these forms and the differential diagnosis of them have been described with the exception of secondary diabetes and diabetes due to syndromes.
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Acknowledgements
All the Authors of this paper are collaborating with the Study Group on Diabetes of Italian Society of Pediatric Endocrinology and Diabetology (ISPED) (Coordinated by Fortunato Lombardo) for diagnostic definition of all Italian cases of non-autoimmune diabetes in children. Dr Angela Napoli is the Coordinator of Italian Diabetes and Pregnancy Study Group of SID- AMD and the next Chair of Diabetes and Pregnancy Study Group (DPSG) of European Association for the Study of Diabetes (EASD). We have a debt of gratitude to Prof Francesco Prisco who is among the most tenacious supporters of the need to understand the pathogenesis of all forms of diabetes in children. We thank Pasquale Villano, Loredana Russo, Olimpia Bitterman, Michele Pinelli, Fabio Acquaviva, Fernanda Iafusco, Paola De Sanctis, Alfonso Galderisi, Roberta Caiazzo, Salvatore Napodano and Linda Sessa for revising and editing the manuscript.
We are pleased to dedicate this Chapter to all the children and adolescents with diabetes and their families who are in follow-up in the ‘G.Stoppoloni’ Centre of the SUN of Naples.
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Iafusco, D. et al. (2017). Not Autoimmune Diabetes Mellitus in Paediatrics. In: Scaramuzza, A., de Beaufort, C., Hanas, R. (eds) Research into Childhood-Onset Diabetes. Springer, Cham. https://doi.org/10.1007/978-3-319-40242-0_12
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DOI: https://doi.org/10.1007/978-3-319-40242-0_12
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