{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,9]],"date-time":"2025-11-09T03:49:23Z","timestamp":1762660163505,"version":"3.37.3"},"reference-count":19,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2022,2,21]],"date-time":"2022-02-21T00:00:00Z","timestamp":1645401600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2022,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Organ laterality defects in primary ciliary dyskinesia (PCD) are common, ranging from complete mirror image organ arrangement, situs inversus totalis (SIT), to situs ambiguus (SA), which falls along the spectrum of situs solitus (SS) and SIT. Targeted investigations for organ laterality defects are not universally recommended in PCD consensus statements. Without investigations beyond chest radiography (CXR), clinically significant defects may go undetected leading to increased morbidity. We hypothesize that clinically significant SA defects remain undetected on CXR and targeted investigations are needed to detect various laterality defects associated with morbidity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This retrospective study collected data from PCD clinics at two Canadian children's hospitals from 2012 to 2020. Participants &lt;30 years old with a confirmed or clinical diagnosis of PCD were enrolled. CXR images were reviewed, and reports of other targeted investigations, including chest computed tomography, abdominal ultrasound, echocardiogram, upper gastrointestinal series, and splenic function studies, were extracted from medical records. Situs classifications from CXR alone versus CXR with add\u2010on targeted investigations were compared using Cochran's <jats:italic>q<\/jats:italic> and McNemar tests.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>One hundred and fifty\u2010nine PCD patients were included, median age at PCD diagnosis of 6.1 years (range: 0\u221228). The situs classification differed significantly from CXR images alone versus CXR with add\u2010on targeted investigations (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001); SS\u00a088 (55%) versus 75 (47%), SIT 59 (37%) versus 46 (29%), and SA 12 (8%) versus 38 (24%). Identified SA defects were cardiovascular (21, 13%), intestinal (9, 6%), and\/or splenic (16,10%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>In PCD patients, clinically significant SA defects may not be detected by CXR alone. Our results suggest that the routine use of CXR with add\u2010on targeted investigations may be justified.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.25853","type":"journal-article","created":{"date-parts":[[2022,2,5]],"date-time":"2022-02-05T07:43:23Z","timestamp":1644047003000},"page":"1318-1324","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Going beyond the chest X\u2010ray:\u00a0Investigating laterality defects in primary ciliary dyskinesia"],"prefix":"10.1002","volume":"57","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4291-3513","authenticated-orcid":false,"given":"Wallace B.","family":"Wee","sequence":"first","affiliation":[{"name":"Respiratory Medicine The Hospital for Sick Children Toronto Ontario Canada"},{"name":"Child Health Evaluative Sciences The Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kimberley R.","family":"Kaspy","sequence":"additional","affiliation":[{"name":"Respiratory Medicine McGill University Health Centre Research Institute Quebec Montreal Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael G.","family":"Sawras","sequence":"additional","affiliation":[{"name":"Translational Medicine The Hospital for Sick Children Toronto Ontario Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael R.","family":"Knowles","sequence":"additional","affiliation":[{"name":"Department of Medicine Marsico Lung Institute University of North Carolina School of Medicine Chapel Hill North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maimoona A.","family":"Zariwala","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Marsico Lung Institute University of North Carolina School of Medicine Chapel Hill North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margaret W.","family":"Leigh","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Marsico Lung Institute University of North Carolina Chapel Hill North Carolina USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2169-9407","authenticated-orcid":false,"given":"Sharon D.","family":"Dell","sequence":"additional","affiliation":[{"name":"Child Health Evaluative Sciences The Hospital for Sick Children Toronto Ontario Canada"},{"name":"Respiratory Medicine, Department of Pediatrics BC Children's Hospital University of British Columbia Vancouver British Columbia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6066-6750","authenticated-orcid":false,"given":"Adam J.","family":"Shapiro","sequence":"additional","affiliation":[{"name":"Respiratory Medicine McGill University Health Centre Research Institute Quebec Montreal Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,2,21]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1084576"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23304"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.106.649038"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.13-1704"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/hrt.41.5.544"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-010-9762-1"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201511-748OC"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201805-0819ST"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201803-0548OC"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.00725-2019"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpheart.01118.2011"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0057545"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2018-212104"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2012.08.016"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10096-010-1049-1"},{"issue":"3","key":"e_1_2_8_17_1","first-page":"146","article-title":"Howell\u2010Jolly 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