INSULIN RESISTANCE AND BETA-CELL FUNCTION IN WERNER SYNDROME (ADULT PROGERIA)

Diabetes mellitus or glucose intolerance has been frequently reported among patients affected by Werner's syndrome. Association with hyperin sulinism suggests a primary insulin resistance. A 34-yr old non-diabet ic lean patient with Werner's syndrome presented chronic painful ulcer ations and atherosclerosis. Glucose tolerance was slightly impaired, w ith basal and post-oral glucose hyperinsulinaemia. First phase insulin secretion after intravenous glucose challenge was within normal range . Peripheral insulin sensitivity and responsiveness was assessed by me ans of a 3-step hyperinsulinaemic clamp. The dose-response curve was b lunted and shifted to the right, suggesting both insulin-binding and p ost-binding defects. This study confirmed the predominance of insulin resistance in Werner's syndrome, even in the absence of diabetes.