MULTIPLE ENDOCRINE NEOPLASIA TYPE-1 IN FRANCE - CLINICAL AND GENETIC-STUDIES

Multiple endocrine neoplasia type 1 (MEN1) is a hereditary syndrome ch aracterized by the involvement of several endocrine glands, including the parathyroid glands, the pancreatic islet cells, the anterior pitui tary gland and other neuroendocrine tissues. In order to build up a Fr ench MEN1 register, a collaborative network was developed through the 'Groupe d'Etude des Neoplasies Endocriniennes Multiples de type 1' or GENEM 1. A 2-year follow-up in 40 medical and surgical units allowed t he identification of more than 150 individual patients and 45 MEN1 fam ilies, and defined the major clinical features of the disease in our s eries, Multiple endocrine neoplasia type 1 is inherited as an autosoma l dominant trait. The gene causing this syndrome has been localized to chromosome 11, band 11q13, and molecular genetic markers nanking the MEN1 locus are of use in identifying disease gene carriers in predispo sed families. Selected data were presented in order to discuss the man agement of patients by combined clinical, biochemical and genetic scre ening, The set-up of a national register by a multi-disciplinary and c ollaborative medical and surgical network will facilitate further rese arch on the clinical management of MEN1 patients and the basic physio- pathology of the disease.