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Heterogeneity of islet pathology in two infants with recent onset diabetes mellitus

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Abstract

The mechanisms by which the beta cells of pancreatic islets are destroyed in insulin-dependent diabetes mellitus (IDDM) are poorly understood. In this report the pancreatic histo- and immunopathology of two children, both HLA-DR 3/4, DQ 2/8 positive and who both died from cerebral oedema within a day of clinical diagnosis of IDDM, were investigated. Patient 1, a 14-month-old girl, had a 4-week history of polydipsia and polyuria. Patient 2, a 3-year-old boy, had 2 days of illness. Both patients had a similarly severe loss of insulin cells but differed markedly as to the extent of lymphocytic islet infiltration (insulitis). Apart from insulitis, marked islet macrophage infiltration was demonstrated in both patients with the HAM-56 monoclonal antibody. Neither patient showed aberrant expression of HLA class II antigens on insulin-immunoreactive cells, but allele-specific HLA-DQ8 expression was evident on endothelial cells. Glutamic acid decarboxylase immunoreactivity was detected in both insulin- and glucagon-immunoreactive cells. It is concluded that the heterogeneity of islet pathology, especially insulitis, may reflect different dynamics and extent rather than different pathomechanisms of immune destruction of islets in IDDM.

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Authors and Affiliations

  1. Department of Medicine, University of Washington, 98195, Seattle, WA, USA

    Å. Lernmark, D. Stenger, D. G. Baskin, J. P. Palmer & L. Li

  2. Department of Pathology, Vrije Universiteit Brussel, Brussels, Belgium

    G. Klöppel

  3. Department of Biological Structure, Veterans Affairs Medical Center, 98108, Seattle, WA, USA

    C. Vathanaprida

  4. Department of Pathology, Malmö General Hospital, Malmö, Sweden

    K. Fält

  5. Department of Medicine, University of Lund, Lund, Sweden

    M. Landin-Olsson

  6. Department of Pathology, University of Washington, 98195, Seattle, WA, USA

    A. M. Gown

  7. Hagedorn Research Laboratory, Gentofte, Denmark

    J. S. Petersen

  8. Department of Pediatrics, Karlskrona Hospital, Karlskrona, Sweden

    H. Edenvall

  9. Department of Pediatrics, University of Washington, 98195, Seattle, WA, USA

    R. S. Mauseth

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  1. Å. Lernmark
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  2. D. Stenger
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  3. D. G. Baskin
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  4. J. P. Palmer
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  5. L. Li
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  6. G. Klöppel
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  7. C. Vathanaprida
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  8. K. Fält
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  9. M. Landin-Olsson
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  13. R. S. Mauseth
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Lernmark, Å., Stenger, D., Baskin, D.G. et al. Heterogeneity of islet pathology in two infants with recent onset diabetes mellitus. Vichows Archiv A Pathol Anat 425, 631–640 (1995). https://doi.org/10.1007/BF00199353

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