Multiple Endocrine Neoplasia(MEN-1) | Japan| PDF | PPT| Case Reports | Symptoms | Treatment

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Multiple Endocrine Neoplasia(MEN-1)

  • 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)

    Primary hyperparathyroidism, gastroenteropancreatic neuroendocrine tumours (GEPNET), and pituitary tumours were seen in 94·4%, 58·6% and 49·6% of patients, respectively. The prevalence of insulinoma was higher in the Japanese than in the West (22%vs 10%). In addition, 37% of patients with thymic carcinoids were women, while most were men in western countries. The MEN1 mutation positive rate was 91·7% in familial cases and only 49·3% in sporadic cases. Eight novel mutations were identified. Despite the availability of genetic testing for MEN1, the application of genetic testing, especially presymptomatic diagnosis for at-risk family members appeared to be insufficient.

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