Going beyond the chest X‐ray: Investigating laterality defects in primary ciliary dyskinesia

Background 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 d...

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Bibliographic Details
Published in:Pediatric pulmonology 2022-05, Vol.57 (5), p.1318-1324
Main Authors: Wee, Wallace B., Kaspy, Kimberley R., Sawras, Michael G., Knowles, Michael R., Zariwala, Maimoona A., Leigh, Margaret W., Dell, Sharon D., Shapiro, Adam J.
Format: Article
Language:English
Subjects:
Adult
bronchiectasis
Canada
Child
Ciliary Motility Disorders
Dyskinesia
epidemiology
Humans
imaging
Kartagener Syndrome - complications
Kartagener Syndrome - diagnostic imaging
primary ciliary dyskinesia
pulmonology
Radiography
Respiration Disorders - complications
Retrospective Studies
Situs Inversus - diagnostic imaging
X-Rays
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Summary:Background 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. Methods This retrospective study collected data from PCD clinics at two Canadian children's hospitals from 2012 to 2020. Participants
ISSN:8755-6863
1099-0496
DOI:10.1002/ppul.25853