MORBIDITY IN TURNER-SYNDROME

Turner syndrome afflicts approximately 50 per 100,000 females and is c haracterized by retarded growth, gonadal dysgenesis, and infertility. Much attention has been focused on growth and growth promoting therapi es, while less is known about the natural course of the syndrome, espe cially in adulthood. We undertook this study to assess the incidence o f diseases relevant in the study of Turner syndrome. The study period was from January 1, 1984 to December 31, 1993, and the study base was all women living in Denmark during the study period. We used data from the Danish Cytogenetic Central Register and the Danish National Regis try of Patients to assess morbidity. This study supports several earli er studies reporting increased morbidity and confirms results of a rec ent study on cancer in Turner syndrome. Women with Turner syndrome see m to have an increased incidence of fractures, osteoporotic fractures in adulthood, and non-osteoporotic fractures in childhood. Furthermore , diabetes mellitus, both NIDDM and IDDM, was found with a markedly in creased incidence in Turner syndrome, as well as ischemic heart diseas e, hypertension and stroke. The risk of cancer, except cancer of the l arge bowel, does not seem to be elevated in Turner syndrome. Our data suggest that patients with Turner syndrome are extraordinarily prone t o abnormalities constituting the metabolic syndrome (e.g., hypertensio n, dyslipidaemia, NIDDM, obesity, hyperinsulinemia and hyperuricemia). The present data may help to explain the decreased life span found in patients with Turner syndrome. (C) 1998 Elsevier Science Inc.