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. 2018 Oct 12:10:1479-1487.
doi: 10.2147/CLEP.S174606. eCollection 2018.

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

Jes Sloth Mathiesen  1   2 , Jens Peter Kroustrup  3 , Peter Vestergaard  3   4 , Kirstine Stochholm  5   6 , Per Løgstrup Poulsen  5 , Åse Krogh Rasmussen  7 , Ulla Feldt-Rasmussen  7 , Sten Schytte  8 , Henrik Baymler Pedersen  9 , Christoffer Holst Hahn  10 , Jens Bentzen  11 , Sören Möller  2   12 , Mette Gaustadnes  13 , Maria Rossing  14 , Finn Cilius Nielsen  14 , Kim Brixen  2 , Anja Lisbeth Frederiksen  2   15 , Christian Godballe  1
Affiliations

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

Jes Sloth Mathiesen et al. Clin Epidemiol. .

Abstract

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.

Keywords: Denmark; incidence; multiple endocrine neoplasia 2A; prevalence.

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Conflict of interest statement

Disclosure The authors report no conflict of interest in this work.

Figures

Figure 1
Figure 1
Flow chart showing identification of MEN2A patients. Dotted boxes indicate methods used and additional MEN2A patients for inclusion. Notes: aCopenhagen University Hospital, Aarhus University Hospital, Odense University Hospital, and Aalborg University Hospital. bDanish MTC cohort, pheochromocytoma cohort, hyperparathyroidism cohort, Hirschsprung cohort, multiple endocrine neoplasia cohort, and lichen amyloidosis cohort. Abbreviations: MEN2A, multiple endocrine neoplasia 2A; RET, Rearranged during Transfection.
Figure 2
Figure 2
Point prevalence of multiple endocrine neoplasia 2A in Denmark at January 1 according to years.
See this image and copyright information in PMC

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