Abstract
Multiple Endocrine Neoplasia syndromes are genetic diseases with au-tosomal dominant inheritance. There are three such syndromes. The MEN type 1 syndrome is characterized by hyperparathyroidism, pancreatic islets tumors, and pituitary adenomas. The genetic defect has been mapped to chromosome 11. The MEN type 2A syndrome includes hyperparathyroidism, medullary thyroid carcinoma and pheochromocytoma(s). The MEN type 2B syndrome includes medullary thyroid carcinoma and pheochromocytoma(s) but also ganglio-neuromas of the gastrointestinal tract. The inherited defects responsible for the MEN2 syndromes map to the pericentromeric region of chromosome 10. Hormonal screening of members of MEN families have led to earlier diagnosis and treatment. This has improved the quality of life and the survival. In our experience the patients with MEN 2A syndromes have the same survival as the normal population. The introduction of genetic diagnosis will further improve the outcome of treatment for these patients.
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