Evaluation of insulin response in glucose tolerance test in a patient withWerner's syndrome: A 16-year follow-up study

To clarify the effect of Werner's syndrome (WS) on beta-islet cell function , the oral glucose tolerance test (OGTT) was repeatedly performed over a pe riod of 16 years in one patient with WS, The data obtained on insulin secre tion were assessed in this study. The patient was a 50-yr-old woman of cons anguineous parentage. She presented with gray hair, cataracts, a beak-shape d nose and high-pitched voice. She was diagnosed as WS on the basis of her characteristic appearance. OGTT was performed 14 times during 9 admissions to our hospital. After ingestion of glucose, plasma glucose (PG) levels and immune-reactive insulin (IRI) at 0, 30, 60, 90, 120 and 180 min were deter mined. PG levels during OGTT gradually increased during dietary therapy and , at the age of 48, insulin treatment was started [PG level at 120 min duri ng OGTT at 46 yr (before treatment) was 1.5 times that at 34 yr], Insulin s ecretion had also gradually decreased during the follow-up period (sum of I RI at 34 yr during OGTT post-treatment; 550.8 IU/ml, sum of IRI at 50 yr du ring OGTT post-treatment; 244.5 IU/ml), However, the insulinogenic indices were maintained at almost the same level value. Our results indicate that i nsufficient insulin secretion, which could not overcome insulin resistance, might play a crucial role in the pathophysiology and progression of diabet es in WS along with insulin resistance due to a post-receptor defect.