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Ketosis-prone diabetes: dissection of a heterogeneous syndrome using an immunogenetic and beta-cell functional classification, prospective analysis, and clinical outcomes

  • Mario Maldonado
  • Christiane S Hampe
  • Lakshmi K Gaur
  • Susana D'Amico
  • Dinakar Iyer
  • Lisa P Hammerle
  • Douglas Bolgiano
  • Lucille Rodriguez
  • Arun Rajan
  • Åke Lernmark
  • Ashok Balasubramanyam
Publishing year: 2003
Language: English
Pages: 5090-5098
Publication/Series: Journal of Clinical Endocrinology and Metabolism
Volume: 88
Issue: 11
Document type: Journal article
Publisher: The Endocrine Society

Abstract english

Ketosis-prone diabetes is heterogeneous. Its causes could include novel beta-cell functional defects. To characterize such defects, 103 patients with diabetic ketoacidosis were evaluated for beta-cell autoimmunity and human leukocyte antigen (HLA) class II alleles, with longitudinal measurements of beta-cell function and biochemical and clinical parameters. They were classified into four A beta groups, based on the presence of glutamic acid decarboxylase (GAD)65, GAD67, or IA-2 autoantibodies (A+ or A-) and beta-cell functional reserve (beta+ or beta-). The group distribution was: 18 A+beta-, 23 A-beta-, 11 A+beta+, and 51 A-beta+. Collectively, the two beta- groups differed from the two beta+ groups in earlier onset and longer duration of diabetes, lower body mass index, less glycemic improvement, and persistent insulin requirement. HLA class II genotyping showed that the A-beta- group differed from the A+beta- group in having lower frequencies of two alleles strongly associated with


  • Endocrinology and Diabetes


  • Diabetes and Celiac Unit
  • ISSN: 1945-7197
E-mail: ake [dot] lernmark [at] med [dot] lu [dot] se

Principal investigator

Diabetes and Celiac Unit

+46 40 39 19 01

+46 70 616 47 79


Jan Waldenströms gata 35, Malmö


Lund University Diabetes Centre, CRC, SUS Malmö, Jan Waldenströms gata 35, House 91:12. SE-214 28 Malmö. Telephone: +46 40 39 10 00