Multiple endocrine neoplasia type 1

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