Abstract
We studied the clinical characteristics of Japanese children with idiopathic type 1 diabetes who had had a remarkably rapid onset, severe metabolic disorder and absolute insulin deficiency at diagnosis. In 85 Japanese children with type 1 diabetes, 14 (16.5%) were classified as idiopathic type 1 diabetes mellitus with no evidence of anti-islet autoantibodies. Among the 14 patients with the idiopathic form, three were identified as having a fulminant onset form. These three patients had had a remarkably rapid onset with a short symptomatic period of less than seven days prior to the onset of overt diabetes. They exhibited severe ketoacidosis and had low levels of HbA1c despite high concentrations of blood glucose at diagnosis. Their initial serum C-peptide levels were extremely low or undetectable. Most of the patients experienced viral infections prior to the onset of the disease. These findings suggest that the nonautoimmune, fulminant onset form of type 1 diabetes may be not rare in the Japanese population. This form is characterized by a remarkably rapid onset with severe metabolic disorder and absolute deficiency of insulin secretion at onset. Viral infections may be associated with the rapid destruction of beta-cells without an autoimmune mechanism.
