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Multiple Endocrine Neoplasia(MEN-1)

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  • Multiple Endocrine Neoplasia(MEN-1)

    Multiple endocrine neoplasia(MEN-1) is also known as Wermer's syndrome. MEN-1 is a hereditory disorder .This type of hereditory disosorder generally passed from one generation to next generation. It affects the hormone producing glands.This disorder increases the development of tumors like cancerous and non cancerous tumors in glands.Non cancerous tumors may develop in parathyroid glands,Neuroendocrine system,anterior pituitory glands and skin. The symptoms are tiredness, bone pain, fractures and kidney stones, as well as stomach or intestinal ulcers.

  • Multiple Endocrine Neoplasia(MEN-1)

    Founder mutations 1466del12 and 1657insC were found in 39 and 29 individuals, and D418N, G156R and R527X mutations in 9, 3 and 2 individuals respectively. Except for pituitary adenoma and nonfunctional pancreatic tumour (NFPT), age was a risk factor for all the disease manifestations. For NFPT, frameshift/nonsense mutations (1657insC, R527X) gave an odds ratio (OR) of 3.26 (95% confidence intervals (CI), 1.27-8.33; P = 0.014) compared with in-frame/missense mutations (1466del12, D418N, G156R); including the founder mutation carriers (n = 68) only, the 1657insC mutation gave an OR of 3.56 (CI, 1.29-9.83; P = 0.015). For gastrinoma, in-frame/missense mutations predicted the risk with an OR of 6.77 (CI, 1.31-35.0; P = 0.022), and in the founder mutations group the 1466del12 mutation gave an OR of 15.09 (CI, 1.73-131.9, P = 0.014).

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