{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,13]],"date-time":"2025-05-13T14:32:39Z","timestamp":1747146759397,"version":"3.40.5"},"reference-count":39,"publisher":"Wiley","issue":"13","license":[{"start":{"date-parts":[[2021,2,9]],"date-time":"2021-02-09T00:00:00Z","timestamp":1612828800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100007542","name":"Agence Fran\u00e7aise de S\u00e9curit\u00e9 Sanitaire des Produits de Sant\u00e9","doi-asserted-by":"publisher","award":["2019 5005"],"award-info":[{"award-number":["2019 5005"]}],"id":[{"id":"10.13039\/501100007542","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Birth Defects Research"],"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>REMERA (REgistre des Malformations En Rh\u00f4ne\u2010Alpes) is a registry of congenital malformations that covers 58,000 births annually in Central\u2010East France. In 2010, the registry raised an alarm to the health authorities (Sant\u00e9 publique France, SpF) about three cases of a unilateral isolated transverse upper limb reduction defect (UITULRD) in a small subarea; the general prevalence of this defect is one case in 10,000 births. In the following years, more infants were born with the same malformation in the same territory of the Ain department. Public health authorities, supported by an expert committee, rejected the existence of a cluster, but we aim here at providing evidence for this cluster.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Geocoded data for all UITULRD cases (ICD\u201010 codes Q71.2 and Q71.3) were extracted from the REMERA database. We conducted a Kulldorff cluster analysis of these data, using the spatial SaTScanTM algorithm.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The analysis found a cluster of eight cases of UITULRD among the 8,204 births occurring between 2009 and 2014 within a circle of 16.24\u2009km radius centered on a village of the Ain department, whereas 0.82 cases were expected under a uniform probability of such a birth throughout the registry territory. This represents an almost 10fold excess over the expected number of cases (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.0057).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The arguments used to deny the cluster are disputed and we present the evidence supporting its reality. The controversy that has followed the alarm has compromised the search for the cause(s) of this excess of rare malformations.<\/jats:p><\/jats:sec>","DOI":"10.1002\/bdr2.1876","type":"journal-article","created":{"date-parts":[[2021,2,11]],"date-time":"2021-02-11T01:17:02Z","timestamp":1613006222000},"page":"1015-1025","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Evidence for a cluster of rare birth defects in the Ain department (France)"],"prefix":"10.1002","volume":"113","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1823-3075","authenticated-orcid":false,"given":"Elisabeth","family":"Gnansia","sequence":"first","affiliation":[{"name":"Registre des Malformations En Rh\u00f4ne\u2010Alpes (REMERA)  Lyon France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lucas","family":"Michon","sequence":"additional","affiliation":[{"name":"REMERA, Hospices Civils de Lyon  Lyon France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emmanuelle","family":"Amar","sequence":"additional","affiliation":[{"name":"REMERA, Hospices Civils de Lyon  Lyon France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacques","family":"Est\u00e8ve","sequence":"additional","affiliation":[{"name":"Hospices Civils de Lyon, Universit\u00e9 Lyon 1, CNRS, UMR 5558  Lyon France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,2,9]]},"reference":[{"key":"e_1_2_8_2_1","first-page":"272","volume-title":"Un silence toxique","author":"Amar E.","year":"2019"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.NAJ.0000481286.16720.6e"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.61875"},{"key":"e_1_2_8_5_1","first-page":"127","volume-title":"Congenital deformities of the upper extremities","author":"Birch\u2010Jensen A.","year":"1949"},{"key":"e_1_2_8_6_1","first-page":"1","article-title":"Guidelines for investigating clusters of health events","volume":"39","author":"CDC","year":"1990","journal-title":"MMWR Recommendations and Reports"},{"key":"e_1_2_8_7_1","first-page":"22","article-title":"Investigating suspected cancer clusters and responding to community concerns. 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