Evidence map›Paper›PMID 41803868›Full record

ArticleBMC medicine2026

PCDSOS: a novel clinical predictive tool for screening primary ciliary dyskinesia in adult bronchiectasis patients-a multicenter derivation and external validation study.

Wangji Zhou, Lin Wang, Qiaoling Chen, Yaqi Wang, Haiyu Pang, Anhui Guo, Aohua Wu, Xueqi Liu, Jinrong Dai, Shuzhen Meng and 8 more

Abstract readMulticenter StudyValidation Study
PubMed Full text at PMC Publisher
In one paragraph

Article in BMC medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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0citing papers in PubMed
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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

18 authors.

Wangji Zhou *Department of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Lin Wang *Department of Pulmonary and Critical Care Medicine, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China.
Qiaoling ChenDepartment of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Yaqi WangDepartment of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Haiyu PangInstitute of Clinical Medicine, National Infrastructures for Translational Medicine, State Key Laboratory of Common Mechanism Research for Major Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Anhui GuoDepartment of Primary Care and Family Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Aohua WuDepartment of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Xueqi LiuDepartment of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Jinrong DaiDepartment of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Shuzhen MengDepartment of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Rongchun WangDepartment of Pulmonary and Critical Care Medicine, The Second People's Hospital of Hunan Province, Hospital of Hunan University of Chinese Medicine, Changsha, Hunan, China.
Danhui YangDepartment of Pulmonary and Critical Care Medicine, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China.
Yaping LiuCenter for Rare Diseases, The State Key Laboratory for Complex, Severe, and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Weiguo ZhuDepartment of Primary Care and Family Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Kai-Feng XuDepartment of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China.
Xue ZhangMcKusick-Zhang Center for Genetic Medicine, State Key Laboratory for Complex Severe and Rare Diseases, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences, School of Basic Medicine, Peking Union Medical College, Beijing, China.
Hong LuoDepartment of Pulmonary and Critical Care Medicine, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China. luohonghuxi@csu.edu.cn.
Xinlun TianDepartment of Pulmonary and Critical Care Medicine, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China. xinlun_t@sina.com.

Funding

Capital's Funds for Health Improvement and Research 2024-1-2093Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences 2023-I2M-C&T-A-002Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences 2025-I2M-C&T-A-001Hunan Provincial Natural Science Foundation of China 2024JJ3040National High-Level Hospital Clinical Research Funding 2022-PUMCH-B-107National High-Level Hospital Clinical Research Funding 2023-PUMCH-E-012National Key Research and Development Program of China 2022YFC2703901National Natural Science Foundation of China 82270048Noncommunicable Chronic Diseases-National Science and Technology Major Project 2024ZD0529602
6 · The paper itself

Abstract

backgroundPrimary ciliary dyskinesia (PCD) is a rare but underdiagnosed genetic cause of adult bronchiectasis, with current predictive tools (e.g., PICADAR, NA-CDCF) primarily validated in children and lacking adult-specific predictors (e.g., subfertility). This study aimed to develop and validate a practical tool (PCDSOS) for PCD screening in adult bronchiectasis.

methodsDerivation group (n = 287) from Peking Union Medical College Hospital (2013-2025) and validation group (n = 107) from The Second Xiangya Hospital (2016-2024) were included. All patients completed ≥ 1 PCD diagnostic test (nasal nitric oxide, whole-exome sequencing, transmission electron microscopy, or high-speed video microscopy analysis). Logistic regression was used to develop PCDSOS, with performance assessed by AUC, calibration curve, and decision curve analysis.

resultsExisting tools showed reduced accuracy in adults (AUC: 0.76-0.85 vs. 0.84-0.98 in original studies). PCDSOS included 6 predictors: pulmonary atelectasis/lobectomy in middle lobe/lingula (P, 2 points), neonatal chest symptoms (C, 2 points), organ laterality defects (D, 5 points), chronic sinusitis (S, 2 points), chronic otitis media/hearing loss from childhood (O, 1 point), and subfertility (S, 1 point). At cutoff = 3, PCDSOS had sensitivity 0.86, specificity 0.76 (derivation cohort, AUC = 0.90) and sensitivity 0.90, specificity 0.67 (validation cohort, AUC = 0.92). A free web-based version of PCDSOS for automated scoring is available to facilitate clinical application.

conclusionsPCDSOS outperforms existing tools in adult bronchiectasis, providing a cost-effective screening strategy to identify patients requiring further PCD diagnostic testing-critical for preventing irreversible lung damage and guiding genetic counseling.

Indexed as

BronchiectasisCiliary Motility DisordersKartagener SyndromeAdultAgedFemaleHumansMaleMiddle AgedPrimary ciliary dyskinesia; Predictive tool; Diagnostic score

Identifiers

PMID41803868
PMCPMC13085268

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