MULTIPLE ENDOCRINE NEOPLASIA TYPE-I

Multiple endocrine neoplasia type I (MEN I) is an autosomal dominant s yndrome comprising parathyroid, pituitary and pancreatic islet cell tu mours. The gene for MEN I has been mapped to 11q 13 and is flanked by markers close enough to construct haplotypes segregating with the defe ctive gene with > 99.5% predictive accuracy. Biochemical screening for disease manifestation can then be restricted to those carrying the ch romosome 11 haplotype incriminated in that family. Because some patien ts may represent new mutations or may be unaware of family history, th e possibility of MEN I should be entertained whenever a patient with a component of the syndrome is encountered.