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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 cystic fibrosis, 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 cystic fibrosis which was confirmed or probably diagnosed at each center from the beginning of the study are made the investigation of the clinical manifestations, sweat test and CFTR mutations 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 | ten years |
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Inclusion Criteria:
Exclusion Criteria:
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Children who was confirmed diagnosis as cystic fibrosis at the certain hospitals (sponsor and collaborators)
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Baoping Xu, 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 |
|---|---|---|---|
| 2570460 | Background | Kerem B, Rommens JM, Buchanan JA, Markiewicz D, Cox TK, Chakravarti A, Buchwald M, Tsui LC. Identification of the cystic fibrosis gene: genetic analysis. Science. 1989 Sep 8;245(4922):1073-80. doi: 10.1126/science.2570460. | |
| 15173476 | Background | Comeau AM, Parad RB, Dorkin HL, Dovey M, Gerstle R, Haver K, Lapey A, O'Sullivan BP, Waltz DA, Zwerdling RG, Eaton RB. Population-based newborn screening for genetic disorders when multiple mutation DNA testing is incorporated: a cystic fibrosis newborn screening model demonstrating increased sensitivity but more carrier detections. Pediatrics. 2004 Jun;113(6):1573-81. doi: 10.1542/peds.113.6.1573. |
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| ID | Term |
|---|---|
| D003550 | Cystic Fibrosis |
| ID | Term |
|---|---|
| D010182 | Pancreatic Diseases |
| D004066 | Digestive System Diseases |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
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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 |
| 1507302 | Background | Nazer HM. Early diagnosis of cystic fibrosis in Jordanian children. J Trop Pediatr. 1992 Jun;38(3):113-5. doi: 10.1093/tropej/38.3.113. |
| 9538604 | Background | Al-Mahroos F. Cystic fibrosis in bahrain incidence, phenotype, and outcome. J Trop Pediatr. 1998 Feb;44(1):35-9. doi: 10.1093/tropej/44.1.35. |
| 9161949 | Background | Yamashiro Y, Shimizu T, Oguchi S, Shioya T, Nagata S, Ohtsuka Y. The estimated incidence of cystic fibrosis in Japan. J Pediatr Gastroenterol Nutr. 1997 May;24(5):544-7. doi: 10.1097/00005176-199705000-00010. |
| 26631874 | Background | Brennan ML, Schrijver I. Cystic Fibrosis: A Review of Associated Phenotypes, Use of Molecular Diagnostic Approaches, Genetic Characteristics, Progress, and Dilemmas. J Mol Diagn. 2016 Jan;18(1):3-14. doi: 10.1016/j.jmoldx.2015.06.010. Epub 2015 Nov 26. |
| D030342 |
| Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D007232 | Infant, Newborn, Diseases |