Fulminant type 1 diabetes: a nationwide survey in Japan.
OBJECTIVE
To describe the clinical and immunologic characteristics of fulminant type 1 diabetes, a novel subtype of type 1 diabetes, we conducted a nationwide survey.


METHODS
History and laboratory data, including islet-related autoantibodies, were examined in 222 patients with fulminant and nonfulminant type 1 diabetes in our hospitals in addition to another 118 patients with fulminant type 1 diabetes located outside our hospitals in Japan.


RESULTS
In our hospitals, of the 222 patients studied, 43 (19.4%) were diagnosed with fulminant type 1 diabetes, 137 (61.7%) were classified as having autoimmune type 1 diabetes, and 42 were type 1 diabetic subjects who were not fulminant and did not have anti-islet antibodies. An additional 118 fulminant patients outside our hospitals were enrolled, making a total of 161 fulminant type 1 diabetic subjects (83 male and 78 female subjects; 14 children/adolescents and 147 adults) identified from all over Japan. (In 2000, the average incidence was three cases per month.) Flu-like symptoms and pregnancy were more frequently observed in the fulminant than in the autoimmune group (P < 0.001). In the fulminant patients, 4.8% were positive for anti-GAD antibodies and none were positive for anti-islet antigen 2 antibodies.


CONCLUSIONS
Fulminant type 1 diabetes is a distinct subtype and accounts for approximately 20% of the ketosis-onset type 1 diabetes cases in Japan. Flu-like symptoms are characteristic of disease onset. Metabolic derangement is more severe in this subtype than in autoimmune type 1 diabetes.
Imagawa
Akihisa
A
Department of Internal Medicine and Molecular Science, Graduate School of Medicine, Osaka University, Suita, Japan.
Hanafusa
Toshiaki
T
Uchigata
Yasuko
Y
Kanatsuka
Azuma
A
Kawasaki
Eiji
E
Kobayashi
Tetsuro
T
Shimada
Akira
A
Shimizu
Ikki
I
Toyoda
Tetsuya
T
Maruyama
Taro
T
Makino
Hideichi
H
D000293:Adolescent; D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D002648:Child; D003625:Data Collection; D003922:Diabetes Mellitus, Type 1 / Q000145:classification / Q000453:epidemiology / Q000276:immunology; D016883:Diabetic Ketoacidosis / Q000453:epidemiology / Q000276:immunology; D005260:Female; D006801:Humans; D006943:Hyperglycemia / Q000453:epidemiology / Q000276:immunology; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011247:Pregnancy; D011379:Prognosis; D012621:Seasons; D012720:Severity of Illness Index
D001323:Autoantibodies