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Incidence and prevalence of multiple endocrine neoplasia 2A in Denmark 1901-2014: a nationwide study

Title: Incidence and prevalence of multiple endocrine neoplasia 2A in Denmark 1901-2014: a nationwide study
Authors: Mathiesen, Jes Sloth; Kroustrup, Jens Peter; Vestergaard, Peter; Stochholm, Kirstine; Poulsen, Per Løgstrup; Rasmussen, Åse Krogh; Feldt-Rasmussen, Ulla; Schytte, Sten; Pedersen, Henrik Baymler; Hahn, Christoffer Holst; Bentzen, Jens; Möller, Sören; Gaustadnes, Mette; Rossing, Maria; Nielsen, Finn Cilius; Brixen, Kim; Frederiksen, Anja Lisbeth; Godballe, Christian
Source: Mathiesen, J S, Kroustrup, J P, Vestergaard, P, Stochholm, K, Poulsen, P L, Rasmussen, Å K, Feldt-Rasmussen, U, Schytte, S, Pedersen, H B, Hahn, C H, Bentzen, J, Möller, S, Gaustadnes, M, Rossing, M, Nielsen, F C, Brixen, K, Frederiksen, A L & Godballe, C 2018, 'Incidence and prevalence of multiple endocrine neoplasia 2A in Denmark 1901-2014: a nationwide study', Clinical epidemiology, vol. 10, pp. 1479-1487. https://doi.org/10.2147/CLEP.S174606
Publication Year: 2018
Collection: Aarhus University: Research
Subject Terms: Denmark; Incidence; Multiple endocrine neoplasia 2A; Prevalence
Description: Background: The incidence and prevalence of multiple endocrine neoplasia 2A (MEN2A) have only been reported once in a nationwide setting. However, it is unclear whether the figures are representative of other populations, as the major component of the syndrome, hereditary medullary thyroid carcinoma (MTC), has been reported as rare in the same country. We conducted a nationwide retrospective cohort study of MEN2A in Denmark from 1901 to 2014, aiming to describe the incidence and prevalence. Methods: This study included 250 unique MEN2A patients born or resident in Denmark before December 31, 2014. Patients were identified through the Danish REarranged during Transfection (RET) cohort, linkage of MEN2A pedigrees, the Danish MTC cohort, a nationwide collaboration of MEN2 centers, cross-checking of other relevant cohorts, and a systematic literature search. Results: The incidence from 1971 to 2000 was 28 (95% CI: 21-37) per million live births per year. Incidence for the specific mutations or for the overall MEN2A group did not change significantly from 1901 to 2014 (P>0.05). Point prevalence at January 1, 2015, was 24 per million (95% CI: 20-28). Conclusion: The incidence and prevalence of MEN2A in Denmark seem higher than those reported in other countries. This is likely explained by the Danish C611Y founder effect. Also, our data indicate no significant change in MEN2A incidence during the last century.
Document Type: article in journal/newspaper
Language: English
ISSN: 1179-1349
Relation: info:eu-repo/semantics/altIdentifier/pmid/30349395; info:eu-repo/semantics/altIdentifier/pissn/1179-1349; info:eu-repo/semantics/altIdentifier/eissn/1179-1349
DOI: 10.2147/CLEP.S174606
Availability: https://pure.au.dk/portal/en/publications/98260e74-2c77-46e0-9ca1-a470043f0d0e; https://doi.org/10.2147/CLEP.S174606; https://www.scopus.com/pages/publications/85057767451; https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6190626/pdf
Rights: info:eu-repo/semantics/openAccess
Accession Number: edsbas.187C5BD9
Database: BASE