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Clinical studies of multiple endocrine neoplasia type 1 (MEN1)
Abstract
Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant disorder characterized by the combined occurrence of parathyroid, pancreatic islet and anterior pituitary tumours. To facilitate a screening programme for MEN1, we investigated 709 people (364 males and 345 females, age range 1-84 years) from 62 MEN1 families, and 36 non-familial MEN1 patients. Of those investigated, 220 (95 males and 125 females, age range 8-79 years) suffered from MEN1. Parathyroid, pancreatic and pituitary tumours occurred in 95%, 41% and 30% of the patients, respectively. Parathyroid tumours were the first manifestation of MEN1 in 87% of patients, and amongst the pituitary and pancreatic tumours, somatotrophinomas and gastrinomas were more common in patients above the age of 40 years, whilst insulinomas occurred more frequently in patients below the age of 40 years. Biochemical screening indicated that the penetrance of MEN1 by the ages of 20, 35 and 50 years was 43%, 85% and 94%, respectively, and that the development of MEN1 was confined to first-degree relatives in 91% of patients and to second-degree relatives in 9% of patients. These findings have helped to define a proposed screening programme for MEN1.
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Citations
Cited by
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Characterization of Mutations in Patients with Multiple Endocrine Neoplasia Type 1
Bassett, J. H. D., Forbes, S. A., Pannett, A. A. J., Lloyd, S. E., Christie, P. T., Wooding, C., Harding, B., Besser, G. M., Edwards, C. R., Monson, J. P., et al. (1998) · The American Journal of Human Genetics · doi:10.1086/301729 · Cited by 444
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Adrenal involvement in multiple endocrine neoplasia type 1
Langer, P., Cupisti, K., Bartsch, D. K., Nies, C., Goretzki, P. E., Rothmund, M., & Röher, H. D. (2002) · World Journal of Surgery · doi:10.1007/s00268-002-6492-4 · Cited by 151
Adrenal involvement in multiple endocrine neoplasia type 1: results of 7 years prospective screening
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Metadata
- Authors
- Trump, D., Farren, B., Wooding, C., Pang, J. T., Besser, G. M., Buchanan, K. D., Edwards, C. R., Heath, D. A., Jackson, C. E., Jansen, S., Lips, K., Monson, J. P., O'Halloran, D., Sampson, J., Shalet, S. M., Wheeler, M. H., Zink, A., & Thakker, R. V.
- Venue
- QJM
- Publisher
- Oxford University Press (OUP)
- Published
- 1996-09-01
- Volume
- 89
- Issue
- 9
- Pages
- 653-670
- ISSN
- 0033-5622 (ISSN-L)
- 1460-2725
- 1460-2393
- OpenAlex
- W1989146112
- Cited by
- 471
- References
- 0
- FWCI
- 13.67
- Type
- journal article
- Language
- en
- Open access
- closed
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