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

Jes Sloth Mathiesen; Jens Peter Kroustrup; Peter Vestergaard; Kirstine Stochholm; Per Løgstrup Poulsen; Åse Krogh Rasmussen; Ulla Feldt-Rasmussen; Sten Schytte; Henrik Baymler Pedersen; Christoffer Holst Hahn; Jens Bentzen; Sören Möller; Mette Gaustadnes; Maria Rossing; Finn Cilius Nielsen; Kim Brixen; Anja Lisbeth Frederiksen; Christian Godballe

doi:10.2147/CLEP.S174606

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

Jes Sloth Mathiesen^*, Jens Peter Kroustrup, Peter Vestergaard, Kirstine Stochholm, Per Løgstrup Poulsen, Åse Krogh Rasmussen, Ulla Feldt-Rasmussen, Sten Schytte, Henrik Baymler Pedersen, Christoffer Holst Hahn, Jens Bentzen, Sören Möller, Mette Gaustadnes, Maria Rossing, Finn Cilius Nielsen, Kim Brixen, Anja Lisbeth Frederiksen, Christian Godballe

^*Corresponding author for this work

Department of Clinical Medicine

10 Citations (Scopus)

30 Downloads (Pure)

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.

Original language	English
Journal	Clinical Epidemiology
Volume	10
Pages (from-to)	1479-1487
Number of pages	9
ISSN	1179-1349
DOIs	https://doi.org/10.2147/CLEP.S174606
Publication status	Published - 2018

Keywords

Denmark
Incidence
Multiple endocrine neoplasia 2A
Prevalence

Access to Document

10.2147/CLEP.S174606Licence: CC BY-NC

Incidence and prevalence of multiple endocrine neoplasia 2A in Denmark 1901–2014: a nationwide studyFinal published version, 415 KBLicence: CC BY-NC

Cite this

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, 10, 1479-1487. https://doi.org/10.2147/CLEP.S174606

Mathiesen, JS, Kroustrup, JP, Vestergaard, P, Stochholm, K, Poulsen, PL, Rasmussen, ÅK, Feldt-Rasmussen, U, Schytte, S, Pedersen, HB, Hahn, CH, Bentzen, J, Möller, S, Gaustadnes, M, Rossing, M, Nielsen, FC, Brixen, K, Frederiksen, AL & 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

@article{ace2d573421145d2af50704ad8145ef2,

title = "Incidence and prevalence of multiple endocrine neoplasia 2a in denmark 1901–2014: A nationwide study",

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",

author = "Mathiesen, {Jes Sloth} and Kroustrup, {Jens Peter} and Peter Vestergaard and Kirstine Stochholm and Poulsen, {Per L{\o}gstrup} and Rasmussen, {{\AA}se Krogh} and Ulla Feldt-Rasmussen and Sten Schytte and Pedersen, {Henrik Baymler} and Hahn, {Christoffer Holst} and Jens Bentzen and S{\"o}ren M{\"o}ller and Mette Gaustadnes and Maria Rossing and Nielsen, {Finn Cilius} and Kim Brixen and Frederiksen, {Anja Lisbeth} and Christian Godballe",

year = "2018",

doi = "10.2147/CLEP.S174606",

language = "English",

volume = "10",

pages = "1479--1487",

journal = "Clinical Epidemiology",

issn = "1179-1349",

publisher = "Dove Medical Press Ltd",

}

TY - JOUR

T1 - Incidence and prevalence of multiple endocrine neoplasia 2a in denmark 1901–2014

T2 - A nationwide study

AU - Mathiesen, Jes Sloth

AU - Kroustrup, Jens Peter

AU - Vestergaard, Peter

AU - Stochholm, Kirstine

AU - Poulsen, Per Løgstrup

AU - Rasmussen, Åse Krogh

AU - Feldt-Rasmussen, Ulla

AU - Schytte, Sten

AU - Pedersen, Henrik Baymler

AU - Hahn, Christoffer Holst

AU - Bentzen, Jens

AU - Möller, Sören

AU - Gaustadnes, Mette

AU - Rossing, Maria

AU - Nielsen, Finn Cilius

AU - Brixen, Kim

AU - Frederiksen, Anja Lisbeth

AU - Godballe, Christian

PY - 2018

Y1 - 2018

N2 - 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.

AB - 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.

KW - Denmark

KW - Incidence

KW - Multiple endocrine neoplasia 2A

KW - Prevalence

U2 - 10.2147/CLEP.S174606

DO - 10.2147/CLEP.S174606

M3 - Journal article

C2 - 30349395

AN - SCOPUS:85057767451

SN - 1179-1349

VL - 10

SP - 1479

EP - 1487

JO - Clinical Epidemiology

JF - Clinical Epidemiology

ER -

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

Abstract

Keywords

Access to Document

Fingerprint

Cite this