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Multiple Endocrine Neoplasia Type 1

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:
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: 535 relevant articles (16 outcomes, 32 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Primary Hyperparathyroidism
2. Neoplasms (Cancer)
3. Neuroendocrine Tumors (Neuroendocrine Tumor)
4. Gastrinoma
5. Primitive Neuroectodermal Tumors (PNET)

Experts

1. Hua, Xianxin: 9 articles (01/2019 - 06/2006)
2. Marx, Stephen J: 9 articles (05/2017 - 01/2003)
3. Brandi, Maria Luisa: 8 articles (07/2021 - 12/2007)
4. Jensen, Robert T: 8 articles (01/2021 - 12/2003)
5. Thakker, Rajesh V: 7 articles (01/2022 - 10/2012)
6. Libutti, Steven K: 7 articles (01/2019 - 12/2003)
7. Stålberg, Peter: 6 articles (01/2021 - 05/2004)
8. Agarwal, Sunita K: 6 articles (10/2020 - 01/2003)
9. Lines, Kate E: 5 articles (01/2022 - 01/2015)
10. Norton, Jeffrey A: 5 articles (01/2022 - 12/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Multiple Endocrine Neoplasia Type 1:
1. CalciumIBA
2. Cinacalcet (Sensipar)FDA Link
01/01/2022 - "At a median age of 10.8 (2.0-14.4) years, 18 patients received cinacalcet for primary HPT (N = 13 inactive CASR mutation, N = 1 CDC73 mutation, N = 1 multiple endocrine neoplasia type 1, N=3 unknown etiology). "
12/01/2013 - "Impact of cinacalcet hydrochloride in clinical management of primary hyperparathyroidism in multiple endocrine neoplasia type 1."
01/01/2012 - "Cinacalcet has been shown to be effective in reducing or normalizing serum calcium levels in several groups of patients with primary hyperparathyroidism (PHPT), including patients with mild to moderate PHPT, intractable PHPT, and parathyroid carcinoma, and in PHPT as a part of multiple endocrine neoplasia type 1. Cinacalcet slightly reduces PTH levels and has no effects on bone mineral density. "
01/01/2012 - "Cinacalcet has been found to be effective in reducing or normalizing serum calcium levels in several groups of PHPT patients, including those with mild-to-moderate PHPT, intractable disease, parathyroid carcinoma and multiple endocrine neoplasia Type 1. Cinacalcet slightly reduces parathyroid hormone levels and has no effect on bone mineral density. "
09/01/2023 - "The most studied topics from past to present are parathyroidectomy, hypercalcemia, parathyroid hormone/adenoma/glands, calcium/calcimimetics, scintigraphy/sestamibi, bone mineral density, ultrasound, vitamin D, osteoporosis, minimally invasive parathyroidectomy, brown tumor, nephrocalcinosis/nephrolithiasis, multiple endocrine neoplasia type 1, normocalcemia, pregnancy, imaging/preoperative imaging, parathyroid cancer/carcinoma, asymptomatic primary hyperparathyroidism, and cinacalcet. "
3. pasireotideIBA
4. Proteins (Proteins, Gene)FDA Link
5. Octreotide (Sandostatin)FDA LinkGeneric
6. Somatostatin (Somatotropin Release-Inhibiting Factor)IBA
7. Ethanol (Ethyl Alcohol)IBA
8. Parathyroid Hormone (Parathormone)IBA
08/01/1995 - "A five-generation kindred (19 affected, two obligate carriers and 20 unaffected) from Oklahoma USA, in which familial benign (hypocalciuric) hypercalcaemia (FBH) was associated with a developmental elevation in serum parathyroid hormone (PTH) levels, has been investigated for linkage to the candidate chromosomal regions 3q21-q24 and 19p13.3, 11q13, and 11p15, to which the genes for FBH, multiple endocrine neoplasia type 1 (MEN1) and PTH have been mapped respectively. "
01/01/2012 - "The recurrence rate is strongly influenced by: 1) the lack of a pre-operative multiple endocrine neoplasia type 1 diagnosis; 2) the surgeon's experience; 3) the timing of surgery; 4) the possibility of performing intra-operative confirmation (histologic examination, rapid parathyroid hormone assay) of the curative potential of the surgical procedure; and, 5) the surgical strategy. "
09/01/2015 - "Ultrasonographic evaluation of parathyroid hyperplasia in multiple endocrine neoplasia type 1: Positive correlation between parathyroid volume and circulating parathyroid hormone concentration."
08/01/2004 - "Treatment of hyperparathyroidism in the setting of multiple endocrine neoplasia type 1 (MEN-1), particularly in the reoperative setting, can be aided by using the rapid intraoperative parathyroid hormone assay to judge the adequacy of parathyroid debulking. "
05/01/2000 - "Intraoperative parathormone measurement in patients with multiple endocrine neoplasia type I syndrome and hyperparathyroidism."
9. Genetic Markers (Genetic Marker)IBA
10. TamoxifenFDA LinkGeneric

Therapies and Procedures

1. Parathyroidectomy
2. Pancreaticoduodenectomy
3. Conservative Treatment
4. Pancreatectomy
5. Therapeutics