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Multiple Endocrine Neoplasia Type 1 (MEN I)

A form of multiple endocrine neoplasia that is characterized by the combined occurrence of tumors in the PARATHYROID GLANDS, the PITUITARY GLAND, and the PANCREATIC ISLETS. The resulting clinical signs include HYPERPARATHYROIDISM; HYPERCALCEMIA; HYPERPROLACTINEMIA; CUSHING DISEASE; GASTRINOMA; and ZOLLINGER-ELLISON SYNDROME. This disease is due to loss-of-function of the MEN1 gene, a tumor suppressor gene (GENES, TUMOR SUPPRESSOR) on CHROMOSOME 11 (Locus: 11q13).
Also Known As:
MEN I; MEN 1; MEN1; MEA I; MEA 1; Multiple Endocrine Neoplasia Type I; Multiple Endocrine Neoplasms Type 1; Neoplasms, Multiple Endocrine Type I; Neoplasia, Multiple Endocrine Type 1; Neoplasms, Multiple Endocrine Type 1; Wermer Syndrome
Networked: 1034 relevant articles (26 outcomes, 79 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Multiple Endocrine Neoplasia Type 1 (MEN I)
2. Primary Hyperparathyroidism
3. Neoplasms (Cancer)
4. Gastrinoma
5. Primitive Neuroectodermal Tumors (PNET)

Experts

1. Hua, Xianxin: 16 articles (01/2014 - 07/2003)
2. Marx, Stephen J: 15 articles (08/2015 - 01/2003)
3. Marx, S J: 12 articles (12/2001 - 01/2000)
4. Thakker, Rajesh V: 11 articles (04/2014 - 06/2002)
5. Jensen, Robert T: 11 articles (11/2013 - 03/2003)
6. Tsukada, Toshihiko: 11 articles (08/2013 - 10/2002)
7. Brandi, Maria Luisa: 10 articles (08/2014 - 06/2002)
8. Calender, Alain: 10 articles (05/2010 - 02/2002)
9. Libutti, Steven K: 9 articles (11/2015 - 11/2003)
10. Klöppel, Günter: 8 articles (05/2015 - 05/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Multiple Endocrine Neoplasia Type 1:
1. GastrinsIBA
2. cinacalcet (Sensipar)FDA Link
3. Octreotide (Sandostatin)FDA LinkGeneric
4. DNA (Deoxyribonucleic Acid)IBA
5. CalciumIBA
6. Glucose (Dextrose)FDA LinkGeneric
7. Ethanol (Ethyl Alcohol)IBA
8. Complementary DNA (cDNA)IBA
9. Genetic Markers (Genetic Marker)IBA
10. SecretinFDA Link

Therapies and Procedures

1. Parathyroidectomy
2. Thymectomy
06/01/2009 - "Several end points may be helpful for future guidelines: (1) earlier detection of Th-NET in MEN1 patients is required; (2) screening of both sexes is necessary; (3) a prospective study comparing MRI vs. CT scan in yearly screening for Th-NET is needed; (4) a reinforced screening program must be established for patients who belong to clustered families; and (5) thymectomies must be performed in specialized centers."
11/01/2008 - "Since transcervical thymectomy does not prevent all MEN1 patients from developing NETT, existing surveillance guidelines for MEN1 should consider CT scan of the thorax on a regular basis."
06/01/2012 - "This suggests that routine preoperative localizing studies to identify ectopic and supernumerary enlarged parathyroid glands is not useful in the majority of patients with MEN1 undergoing bilateral neck exploration and subtotal parathyroidectomy with cervical thymectomy."
04/01/2014 - "Patients with a preoperative diagnosis of MEN1 were more likely to undergo a complete initial operation (≥ 3 gland parathyroidectomy with transcervical thymectomy, 13/22, 59% vs. 0/4, 0%; P=0.03) and less likely to have recurrent disease (10/22, 45% vs. 3/4, 75%; P<0.001) during follow up than patients diagnosed postoperatively. "
09/01/2004 - "According to our results, tPTX+T (total parathyroidectomy+thymectomy+autotransplantation) seems to be a more favorable surgical approach in patients with MEN1 pHPT than sDE (selective gland exstirpation) and stPTX (subtotal parathyroidectromy) without cervical thymectomy, because recurrences or persistence of the disease are rare. "
3. Gastrectomy
4. Pancreatectomy
5. Pancreaticoduodenectomy