Knapp Emily A, Fink Aliza K, Goss Christopher H, Sewall Ase, Ostrenga Josh, Dowd Christopher, Elbert Alexander, Petren Kristofer M, Marshall Bruce C
1 Cystic Fibrosis Foundation, Bethesda, Maryland.
2 Department of Medicine, University of Washington, Seattle, Washington; and.
Ann Am Thorac Soc. 2016 Jul;13(7):1173-9. doi: 10.1513/AnnalsATS.201511-781OC.
The Cystic Fibrosis Foundation Patient Registry (CFFPR) is an ongoing patient registry study that collects longitudinal demographic, clinical, and treatment information about persons with cystic fibrosis (CF) in the United States. CF is a life-shortening genetic disorder that occurs in approximately 1 in 3,500 births in the United States. High-quality observational data is important for clinical research, quality improvement, and clinical management.
To describe the data collection, patient population, and key limitations of the CFFPR.
Inclusion criteria for the CFFPR include diagnosis with CF or a CFTR-associated disorder, care at an accredited care center program, and provision of informed consent. Data from clinic visits and hospitalizations are collected through a secure website. Loss to follow-up and generalizability were examined using several methods. The accuracy of CFFPR data was evaluated with an audit of 2012 CFFPR data compared to the medical record.
Since 1986, the CFFPR contains the records of 48,463 individuals with CF. Participation among individuals seen at accredited care centers is high, and loss to follow-up is low. An audit of 2012 CFFPR data suggests that the CFFPR contains 95% of clinic visits and 90% of hospitalizations found in the medical record for these patients, and nearly all of the audited fields were highly accurate.
Registries such as the CFFPR are important tools for research, clinical care, and tracking incidence, mortality and population trends.
囊性纤维化基金会患者注册库（CFFPR）是一项正在进行的患者注册库研究，收集美国囊性纤维化（CF）患者的纵向人口统计学、临床和治疗信息。CF是一种缩短寿命的遗传性疾病，在美国约每3500例出生中就有1例发生。高质量的观察性数据对于临床研究、质量改进和临床管理至关重要。
描述CFFPR的数据收集、患者群体及主要局限性。
CFFPR的纳入标准包括诊断为CF或CFTR相关疾病、在经认可的护理中心项目接受护理以及提供知情同意书。门诊和住院数据通过安全网站收集。采用多种方法检查失访情况和可推广性。通过将2012年CFFPR数据与病历进行比对审核来评估CFFPR数据的准确性。
自1986年以来，CFFPR包含了48463名CF患者的记录。在经认可的护理中心就诊的患者参与度高，失访率低。对2012年CFFPR数据的审核表明，CFFPR包含了这些患者病历中95%的门诊就诊记录和90%的住院记录，且几乎所有审核字段都高度准确。
像CFFPR这样的注册库是研究、临床护理以及追踪发病率、死亡率和人群趋势的重要工具。
