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Original Article
Volume 342:301-307 February 3, 2000 Number 5
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A Novel Subtype of Type 1 Diabetes Mellitus Characterized by a Rapid Onset and an Absence of Diabetes-Related Antibodies
Akihisa Imagawa, M.D., Toshiaki Hanafusa, M.D., Ph.D., Jun-ichiro Miyagawa, M.D., Ph.D., Yuji Matsuzawa, M.D., Ph.D., for The Osaka IDDM Study Group

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ABSTRACT

Background and Methods Type 1 diabetes mellitus is now classified as autoimmune (type 1A) or idiopathic (type 1B), but little is known about the latter. We classified 56 consecutive Japanese adults with type 1 diabetes according to the presence or absence of glutamic acid decarboxylase antibodies (their presence is a marker of autoimmunity) and compared their clinical, serologic, and pathological characteristics.

Results We divided the patients into three groups: 36 patients with positive tests for serum glutamic acid decarboxylase antibodies, 9 with negative tests for serum glutamic acid decarboxylase antibodies and glycosylated hemoglobin values higher than 11.5 percent, and 11 with negative tests for serum glutamic acid decarboxylase antibodies and glycosylated hemoglobin values lower than 8.5 percent. In comparison with the first two groups, the third group had a shorter mean duration of symptoms of hyperglycemia (4.0 days), a higher mean plasma glucose concentration (773 mg per deciliter [43 mmol per liter]) in spite of lower glycosylated hemoglobin values, diminished urinary excretion of C peptide, a more severe metabolic disorder (with ketoacidosis), higher serum pancreatic enzyme concentrations, and an absence of islet-cell, IA-2, and insulin antibodies. Immunohistologic studies of pancreatic-biopsy specimens from three patients with negative tests for glutamic acid decarboxylase antibodies and low glycosylated hemoglobin values revealed T-lymphocyte–predominant infiltrates in the exocrine pancreas but no insulitis and no evidence of acute or chronic pancreatitis.

Conclusions Some patients with idiopathic type 1 diabetes have a nonautoimmune, fulminant disorder characterized by the absence of insulitis and of diabetes-related antibodies, a remarkably abrupt onset, and high serum pancreatic enzyme concentrations.


Source Information

From the Department of Internal Medicine and Molecular Science, Graduate School of Medicine, Osaka University, Osaka, Japan.

Address reprint requests to Dr. Imagawa at the Department of Internal Medicine and Molecular Science, Graduate School of Medicine, B5, Osaka University, 2-2 Yamadaoka, Suita 565-0871, Japan, or at imagawa{at}imed2.med.osaka-u.ac.jp.

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Related Letters:

A Novel Subtype of Type 1 Diabetes Mellitus
Honeyman M. C., Coulson B. S., Harrison L. C., Tanaka S., Kobayashi T., Momotsu T., Imagawa A., Miyagawa J.-i., Hanafusa T.
Extract | Full Text  
N Engl J Med 2000; 342:1835-1837, Jun 15, 2000. Correspondence

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