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| Name | Class |
|---|---|
| Shengjing Hospital | OTHER |
| Capital Institute of Pediatrics, China | OTHER |
| Shanghai Children's Medical Center | OTHER |
| Shenzhen Children's Hospital |
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This study is a multicenter, prospective cohort study of patients diagnosed with primary ciliary dyskinesia, the clinical information of recruited patients, including clinical manifestations, lung function, chest imaging, quality of life and other indicators, will be followed for 10 years.
All new cases of primary ciliary dyskinesia which was confirmed or diagnosed at each center from the beginning of the study are made the investigation of the clinical manifestations and specific tests by the standard diagnostic process.Then all the patients' following clinical data will be followed for 10 years (once per six month): clinical manifestations, lung function, chest imaging (once per year), quality of life and other indicators.
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| Measure | Description | Time Frame |
|---|---|---|
| Change from baseline in lung function on the spirometry | forced expiratory volume at one second (FEV1) in Liter | 10 years |
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Inclusion Criteria: A included patient must be coincident with all the following items:
Exclusion Criteria: Subject will be excluded if she or he has one of the following:
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Children who was confirmed diagnosis as primary ciliary dyskinesia at the certain hospitals (sponsor and collaborators)
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Baoping Xu, MD, PhD | Contact | 861059616308 | xubaopingbch@163.com |
| Name | Affiliation | Role |
|---|---|---|
| Kunling Shen, MD, PhD | Beijing Children's Hospital of Capital Medical University, China;China National Clinical Research Center for Respiratory Diseases | Principal Investigator |
| Baoping Xu, MD, PhD | Beijing Children's Hospital of Capital Medical University, China;China National Clinical Research Center for Respiratory Diseases |
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| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 25580864 | Background | Liu Y, Wang L, Tian X, Xu KF, Xu W, Li X, Yue C, Zhang P, Xiao Y, Zhang X. Characterization of gene mutations and phenotypes of cystic fibrosis in Chinese patients. Respirology. 2015 Feb;20(2):312-8. doi: 10.1111/resp.12452. Epub 2015 Jan 8. | |
| 22232363 | Background | Hogg C, Bush A. Genotyping in primary ciliary dyskinesia: ready for prime time, or a fringe benefit? Thorax. 2012 May;67(5):377-8. doi: 10.1136/thoraxjnl-2011-201320. Epub 2012 Jan 9. No abstract available. |
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| ID | Term |
|---|---|
| D002925 | Ciliary Motility Disorders |
| ID | Term |
|---|---|
| D012140 | Respiratory Tract Diseases |
| D010038 | Otorhinolaryngologic Diseases |
| D000072661 | Ciliopathies |
| D000015 | Abnormalities, Multiple |
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| OTHER_GOV |
| The First Affiliated Hospital of Xiamen University | OTHER |
| First Affiliated Hospital of Guangxi Medical University | OTHER |
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| Principal Investigator |
| 26418604 | Background | Shapiro AJ, Zariwala MA, Ferkol T, Davis SD, Sagel SD, Dell SD, Rosenfeld M, Olivier KN, Milla C, Daniel SJ, Kimple AJ, Manion M, Knowles MR, Leigh MW; Genetic Disorders of Mucociliary Clearance Consortium. Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review. Pediatr Pulmonol. 2016 Feb;51(2):115-32. doi: 10.1002/ppul.23304. Epub 2015 Sep 29. |
| 14656747 | Background | Noone PG, Leigh MW, Sannuti A, Minnix SL, Carson JL, Hazucha M, Zariwala MA, Knowles MR. Primary ciliary dyskinesia: diagnostic and phenotypic features. Am J Respir Crit Care Med. 2004 Feb 15;169(4):459-67. doi: 10.1164/rccm.200303-365OC. Epub 2003 Dec 4. |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D030342 | Genetic Diseases, Inborn |