{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,27]],"date-time":"2026-07-27T22:49:21Z","timestamp":1785192561250,"version":"3.55.0"},"reference-count":18,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,10,16]],"date-time":"2023-10-16T00:00:00Z","timestamp":1697414400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Pulmonology"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Structural lung changes seen on computed tomography (CT) scans in persons with primary ciliary dyskinesia (pwPCD) are currently described using cystic fibrosis (CF) derived scoring systems. Recent work has shown structural changes and frequencies that are unique to PCD, indicating the need for a unique PCD\u2010derived\u00a0scoring system.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Chest CT scans from 30 pwPCD, were described for structural changes including bronchiectasis, bronchial wall thickening, mucous plugging, atelectasis, air trapping, and interlobar septal thickening and, additionally, changes previously described as being frequent in pwPCD including extensive tree\u2010in\u2010bud pattern of mucous plugging, bronchoceles or nodules, thickening of interlobar and interlobular septa and whole lobe atelectasis. Based on these findings a novel and unique scoring system, the Specific PCD Evaluation by CT (SPEC) score was constructed. Scans were then re\u2010scored using the SPEC score and results compared to corresponding measurements of lung function to assess structure\u2010function correlation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Total SPEC scores ranged from 0 to 60 (max possible score 90). There was a strong negative correlation between the SPEC score (SPEC) and forced vital capacity (FVC), forced expiratory volume over 1\u2009s\u00a0(FEV<jats:sub>1<\/jats:sub>) and FEV<jats:sub>1<\/jats:sub>\/FVC ratio (\u2212<jats:italic>r<\/jats:italic>\u2009=\u2009.784, \u2212.865, \u2212.872 respectively).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Using PCD\u2010derived\u00a0data we describe the construct of a PCD\u2010specific\u00a0score for assessing lung structural damage on CT scans, the SPEC score. A strong correlation between the SPEC score and PFT variables was identified. The SPEC score holds the potential for describing longitudinal changes in CT scans and assessing the efficacy of interventive therapies in patients with PCD.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26709","type":"journal-article","created":{"date-parts":[[2023,10,16]],"date-time":"2023-10-16T11:13:24Z","timestamp":1697454804000},"page":"72-80","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["The SPEC score\u2014A\u00a0quantifiable CT scoring system for primary ciliary dyskinesia"],"prefix":"10.1002","volume":"59","author":[{"given":"Tarika","family":"Chowdhary","sequence":"first","affiliation":[{"name":"Departments of Respiratory and Sleep Medicine and Medical Imaging Royal Children's Hospital Parkville Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Bracken","sequence":"additional","affiliation":[{"name":"Department of Paediatrics University of Melbourne Parkville Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucy","family":"Morgan","sequence":"additional","affiliation":[{"name":"Department\u00a0of Respiratory Medicine Concord Hospital Sydney Australia"},{"name":"Respiratory Medicine, Concord Repatriation Hospital University of Sydney Sydney Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2000-8910","authenticated-orcid":false,"given":"Andre","family":"Schultz","sequence":"additional","affiliation":[{"name":"Division of Paediatrics School of Medicine, The University of Western Australia Perth Australia"},{"name":"Wal\u2010Yan Centre for Respiratory Health Research Telethon Kids Institute, University of Western Australia Perth Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6129-9075","authenticated-orcid":false,"given":"Phil","family":"Robinson","sequence":"additional","affiliation":[{"name":"Departments of Respiratory and Sleep Medicine and Medical Imaging Royal Children's Hospital Parkville Australia"},{"name":"Department of Paediatrics University of Melbourne Parkville Australia"},{"name":"Respiratory Research Group, Infection and Immunity Theme Murdoch Children's Research Institute Parkville Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,10,16]]},"reference":[{"key":"e_1_2_14_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(21)00453-7"},{"key":"e_1_2_14_3_1","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01090-2016"},{"key":"e_1_2_14_4_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2013-304831"},{"key":"e_1_2_14_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2014-206578"},{"key":"e_1_2_14_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s002470050684"},{"key":"e_1_2_14_7_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.179.3.2027992"},{"key":"e_1_2_14_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.24249"},{"key":"e_1_2_14_9_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.07-2812"},{"key":"e_1_2_14_10_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201908-1590ST"},{"key":"e_1_2_14_11_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.05.00035205"},{"key":"e_1_2_14_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.22577"},{"key":"e_1_2_14_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.21569"},{"key":"e_1_2_14_14_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202206-524OC"},{"key":"e_1_2_14_15_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.13-1162"},{"key":"e_1_2_14_16_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40248-018-0139-2"},{"key":"e_1_2_14_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/jpc.15818"},{"key":"e_1_2_14_18_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.97.10102376"},{"key":"e_1_2_14_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2002.tb03299.x"}],"container-title":["Pediatric Pulmonology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ppul.26709","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,12,29]],"date-time":"2023-12-29T13:12:56Z","timestamp":1703855576000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ppul.26709"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,10,16]]},"references-count":18,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2024,1]]}},"alternative-id":["10.1002\/ppul.26709"],"URL":"https:\/\/doi.org\/10.1002\/ppul.26709","archive":["Portico"],"relation":{},"ISSN":["8755-6863","1099-0496"],"issn-type":[{"value":"8755-6863","type":"print"},{"value":"1099-0496","type":"electronic"}],"subject":[],"published":{"date-parts":[[2023,10,16]]},"assertion":[{"value":"2023-02-12","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-09-19","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-10-16","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}