{"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":1785192561728,"version":"3.55.0"},"reference-count":56,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2022,11,2]],"date-time":"2022-11-02T00:00:00Z","timestamp":1667347200000},"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":[[2023,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Primary ciliary dyskinesia is a rare genetic disorder characterized by recurrent sinopulmonary infections and worsening obstructive lung disease. Kidney and brain involvement is less common and is associated with overlapping ciliopathies\/syndromes. The lungs are impacted early in the course of the disease, so it is vital to monitor lung function and recognize any decline by doing appropriate lung function tests. This systematic review compares different lung function tests and analyzes which one becomes abnormal earlier in the disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A systematic review was conducted following the methodology in the \u201cCochrane Handbook on Systematic Reviews for diagnostic tests.\u201d The Preferred Reporting Items for Systematic Review and Meta\u2010Analyses were used to report the results. The risk of bias assessment was done using \u201cThe Cochrane Handbook for Systematic Reviews tool for interventional studies.\u201d A meta\u2010analysis was not performed due to the small sample size. All studies were analyzed by using Joanna Briggs Institute's critical appraisal tool.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>After screening for the duplication of results and applying inclusion and exclusion criteria, 14 studies were assessed by reading the full texts. Out of these, eight were finally included in this systematic review. The total sample size from all studies was 165, including 80 males. All the studies used spirometry as a lung function test, whereas multiple breath washout was used in five studies. Other tests used for comparison were computed tomography\u00a0(CT),\u00a0magnetic resonance imaging\u00a0(MRI), cardiopulmonary exercise testing,\u00a06\u2010min walk test, DLCO,\u00a0maximal inspiratory pressure,\u00a0maximal expiratory pressure, and PaO<jats:sub>2<\/jats:sub>.\u00a0Lung clearance index\u00a0(LCI)\u00a0by multiple breath washout had a stronger association with the structural changes on CT\/MRI than spirometry indices like forced expiratory volume\u00a0in 1\u2009s (FEV1)\u00a0and forced expiratory flow\u00a0at 25% to 75% of lung volume\u00a0(FEF 25\u201375).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Based on the evidence from this systematic review, LCI becomes abnormal earlier than FEV1 or FEF 25\u201375 and positively correlates with the findings on high\u2010resolution\u00a0CT. It has limitations like the lack of reference values and a complex technique to perform the test.<\/jats:p><\/jats:sec>","DOI":"10.1002\/ppul.26213","type":"journal-article","created":{"date-parts":[[2022,10,21]],"date-time":"2022-10-21T12:10:24Z","timestamp":1666354224000},"page":"475-483","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["In children with primary ciliary dyskinesia, which type of lung function test is the earliest determinant of decline in lung health: A systematic review"],"prefix":"10.1002","volume":"58","author":[{"given":"Adnan","family":"Zafar","sequence":"first","affiliation":[{"name":"John Hopkins Aramco Healthcare Dhahran Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Hall","sequence":"additional","affiliation":[{"name":"University of Southampton Southampton United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,11,2]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2213-2600(21)00453-7"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00176608"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201511-748OC"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2014-0808"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00001010"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1089\/ped.2011.0099"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200811-1731OC"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijpam.2021.03.002"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms22158272"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1136\/thoraxjnl-2011-200301"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201311-2047OC"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajhg.2018.10.020"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.202007-816OC"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201409-1672OC"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201605-331PS"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.4104\/pcrj.2013.00042"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2013.05.003"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.163.3.2002054"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1136\/thorax.57.12.1021"},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1183\/20734735.0016-2021"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005382-200110000-00007"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00247-005-0027-8"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1378\/chest.07-2812"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.21569"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201304-0800OC"},{"key":"e_1_2_11_27_1","unstructured":"Centre for Reviews and Dissemination. 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