Journal of Clinical Endocrinology & Metabolism, Vol 81, 1267-1272, Copyright © 1996 by Endocrine Society
Diabetes mellitus in cystic fibrosis is characterized by islet amyloidosis
M Couce, TD O'Brien, A Moran, PC Roche and PC Butler
Endocrine Research Unit, Mayo Clinic, Rochester, Minnesota 55905, USA.
A high proportion of patients with cystic fibrosis (CF) develop diabetes
mellitus. In common with type II diabetes mellitus, diabetes mellitus in CF
is characterized by a progressive decline in beta-cell function and an
approximately 50% decline in beta-cell mass. It is not known whether islet
amyloidosis (characteristic of type II diabetes mellitus) is present in
diabetes mellitus complicating CF. To address this, pancreatic samples were
obtained at autopsy from 9 control cases (without CF) and 41 cases of CF
that were, in turn, subdivided into 13 nondiabetic, 12 borderline diabetic,
and 16 diabetic cases based on clinical criteria. Islet amyloid was
detected by light microscopy in 69% cases of CF with diabetes mellitus, 17%
of cases with borderline diabetes mellitus, and none of the nondiabetic
cases. Islet amyloid was not present in any of the control cases. Islet
amyloidosis derived from islet amyloid polypeptide is a characteristic
feature of diabetes mellitus in CF as well as type II diabetes mellitus.