Abstract
Established as a novel subtype of type 1 diabetes in Japan in the year 2000, fulminant type 1 diabetes is characterized by a rapid onset and an absence of diabetes-related antibodies. This disease has been mainly reported in East Asia, especially in Japan. The prevalence of fulminant type 1 diabetes among ketosis-onset type 1 diabetes was 19.4% in a Japanese nationwide survey. Both genetic and environmental factors are involved in the etiology of fulminant type 1 diabetes. The former includes, for example, HLA-DR/DQ or CTLA-4 polymorphisms, and the latter includes viral infection. Fulminant type 1 diabetes is the most severe form of diabetes because of the complete loss of pancreatic beta cells at disease onset, and it is also a high-risk subgroup for diabetic microangiopathy.
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