{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,17]],"date-time":"2026-07-17T15:03:45Z","timestamp":1784300625231,"version":"3.55.0"},"reference-count":23,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,5,6]],"date-time":"2026-05-06T00:00:00Z","timestamp":1778025600000},"content-version":"vor","delay-in-days":5,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Birth Defects Research"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Sirenomelia is a rare congenital condition most notably characterized by a single lower limb. Previous studies have suggested a prevalence of approximately 1 per 100,000 births. However, in Wales 17 cases were recorded between 1998 and 2016, suggesting a higher rate of sirenomelia in this country.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>This study compared current prevalence of sirenomelia in Wales with European data. This study further reviewed detailed time, place, and person data on sirenomelia cases in Wales to investigate possible causal factors.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Method<\/jats:title>\n                    <jats:p>\n                      A retrospective cohort study from birth defect surveillance programs. Individual\u2010level records for all welsh cases were examined for evidence of causal factors. Comparator data from other countries were obtained from\n                      <jats:styled-content style=\"fixed-case\">EUROCAT<\/jats:styled-content>\n                      (a European network of population\u2010based registries for the epidemiological surveillance of congenital anomalies).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      European data from 24 national and regional registries of congenital anomalies included 97 cases of sirenomelia\u00a0identified across 9.6 million births between 1998 and 2016, giving a prevalence rate of 1 per 100,000 (95%\n                      <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>\n                      0.83, 1.23). Five regions reported statistically significantly higher rates than that recorded across all registries, while one region reported a significantly lower rate. Small numbers of cases limit definitive statistical interpretation. Analysis of the Wales data did not identify common epidemiological factors between cases.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      The contemporary prevalence of sirenomelia across Europe is consistent with earlier studies at approximately 1 per 100,000 births (95%\n                      <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>\n                      0.83, 1.23). However, there is greater variation between regions than would be expected by chance. There remains no definitive evidence for causal environmental factors.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/bdr2.70051","type":"journal-article","created":{"date-parts":[[2026,5,6]],"date-time":"2026-05-06T10:44:27Z","timestamp":1778064267000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Sirenomelia: A Review of European Prevalence Data and Epidemiological Analysis of 17 Cases Registered in Wales"],"prefix":"10.1002","volume":"118","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6112-7644","authenticated-orcid":false,"given":"Chris","family":"Emmerson","sequence":"first","affiliation":[{"name":"Public Health Wales  Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Olson","sequence":"additional","affiliation":[{"name":"Public Health Wales  Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ceri","family":"Williams","sequence":"additional","affiliation":[{"name":"Public Health Wales  Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9139-8602","authenticated-orcid":false,"given":"Robert","family":"Maddison","sequence":"additional","affiliation":[{"name":"Public Health Wales  Cardiff UK"},{"name":"Wales Gene Park, Wales Genomic Health Centre Cardiff University  Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Tucker","sequence":"additional","affiliation":[{"name":"Public Health Wales Singleton Hospital  Swansea UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4662-7701","authenticated-orcid":false,"given":"Llion","family":"Davies","sequence":"additional","affiliation":[{"name":"Public Health Wales  Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annie","family":"Perraud","sequence":"additional","affiliation":[{"name":"European Commission, Joint Research Centre (JRC)  Ispra Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Bailey","sequence":"additional","affiliation":[{"name":"Public Health Wales  Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ciar\u00e1n","family":"Humphreys","sequence":"additional","affiliation":[{"name":"Public Health Wales  Carmarthen UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rhian","family":"Hughes","sequence":"additional","affiliation":[{"name":"Public Health Wales  Carmarthen UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanna","family":"Sichitiu","sequence":"additional","affiliation":[{"name":"Department of Woman\u2010Mother\u2010Child University Medical Center CHUV  Lausanne 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HSE South (Cork &amp; Kerry), St. Finbarr's Hospital  Cork Ireland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Perthus","sequence":"additional","affiliation":[{"name":"CEMC\u2014Auvergne  Chamalieres Cedex France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Rankin","sequence":"additional","affiliation":[{"name":"Institute of Health &amp; Society Newcastle University  Newcastle upon Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anke","family":"Rissmann","sequence":"additional","affiliation":[{"name":"Malformation Monitoring Centre Saxony\u2010Anhalt, Medical Faculty Otto\u2010von\u2010Guericke University  Magdeburg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florence","family":"Rouget","sequence":"additional","affiliation":[{"name":"Brittany Registry of Congenital Malformations, CHU Rennes, Univ Rennes, Inserm, EHESP, Irset (Institut de recherche en 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