Not Autoimmune Diabetes Mellitus in Paediatrics

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.