Two Cases with Nonautoimmune Type 1 Diabetes Resembling to Fulminant Diabetes

Abstract

Type 1 diabetes is divided into two subtypes: autoimmune and idiopathic diabetes (1). Recently, a novel subtype of idiopathic type 1 diabetes, called fulminant diabetes has been proposed. These patients are characterized with negative islet related auto antibodies, relatively low glycosylated hemoglobin values, and high serum exocrine pancreatic enzyme concentration at the time of diagnosis. The etiology remains to be uncertain. Most of the reported patients were from Japan. These findings suggest that fulminant type1 diabetes may be specific for Japanese patients. We presented two cases; the first case presented with ketoacidosis with slightly high amlylase level after steroid withdrawal. The other case who presented with ketoacidosis had one month history of diabetic symptoms. Both of the patients had family history of diabetes in the first relatives and both of them have negative insulin, islet-cell, glutamic acid decarboxlase (GAD) autoantibodies and low c-peptide levels. We suggest that genetic susceptibility and environmental factors may have influenced the clinical presentation of this form of diabetes. Even though both of the patients have a acute clinical onset of non autoimmune type 1 diabetes the clinical and biochemical signs of the patients did not full fit diagnostic criteria of the fulminant diabetes. Our understanding of the pathogenesis of the disease may improve with more cases reported in the white population.