Genetically Programmed Selective Islet β-Cell Loss in Diabetic Subjects With Wolfram's Syndrome

Avraham Karasik; Carl O'Hara; S Srikanta; Michael Swift; J Stuart Soeldner; C Ronald Kahn; Raymonde D Herskowitz

doi:10.2337/diacare.12.2.135

Original Articles

Genetically Programmed Selective Islet β-Cell Loss in Diabetic Subjects With Wolfram's Syndrome

Avraham Karasik, MD,
Carl O'Hara, MD,
S Srikanta, MD,
Michael Swift, MD,
J Stuart Soeldner, MD,
C Ronald Kahn, MD and
Raymonde D Herskowitz, MD

Joslin Clinic and the E.P. Joslin Research Laboratory, Joslin Diabetes Center, Department of Medicine, Brigham and Women's Hospital, New England Deaconess Hospital, Harvard Medical School Boston, Massachusetts Department of Medicine, University of North Carolina Chapel Hill, North Carolina

Address correspondence and reprint requests to R.D. Herskowitz, MD, Joslin Diabetes Center, One Joslin Place, Boston, MA 02215.

Diabetes Care 1989 Feb; 12(2): 135-138. https://doi.org/10.2337/diacare.12.2.135

Abstract

Insulin-producing (5-cells were selectively absent from the islets of Langerhans in postmortem specimens from two patients with Wolfram's syndrome. In families with multiple cases of this syndrome, we found a very high concordance rate (r = .910, P < .001) among siblings for age at onset of diabetes mellitus. Taken together with the lack of markers for an autoimmune process, these findings suggest that diabetes mellitus in this syndrome results from genetically programmed selective p-cell death.