Buzzetti R, Salvatore D, Baldo E, Forneris M P, Lucidi V, Manunza D, Marinelli I, Messore B, Neri A S, Raia V, Furnari M L, Mastella G
Italian Cystic Fibrosis Research Foundation, Verona, Italy.
J Cyst Fibros. 2009 Jul;8(4):229-37. doi: 10.1016/j.jcf.2009.04.001. Epub 2009 May 6.
Patient registries are organized systems of data collection for scientific, clinical or health strategy purposes. Aims of our review were to document scientific literature based on data and information from cystic fibrosis (CF) registries; to understand which clinical problems have been addressed and for which of these the studies concerned have correctly answered the questions raised (i.e. a methodological critique) and to identify clinical issues in need of further investigation. The review included primary studies starting from a formally constituted CF registry of at least national level, using data from the registry to evaluate research hypotheses. This article is an overview of the research undertaken, focusing in detail on the issues of mortality and survival. The studies considered here focused mainly or secondarily on survival in CF, the aim being to ascertain an improving trend, identify any prognostic factors and, in some cases, attempt to provide a predictive model of survival.
患者登记系统是为科学、临床或健康战略目的而组织的数据收集系统。我们本次综述的目的是基于囊性纤维化（CF）登记处的数据和信息记录科学文献；了解哪些临床问题已得到解决，以及相关研究对所提出的问题给出了正确答案的情况（即方法学批判），并确定需要进一步研究的临床问题。该综述纳入了至少从国家级正式建立的CF登记处开展的初步研究，利用登记处的数据来评估研究假设。本文是对所开展研究的概述，详细聚焦于死亡率和生存率问题。这里所考虑的研究主要或次要关注CF患者的生存情况，目的是确定改善趋势、识别任何预后因素，并且在某些情况下尝试提供生存预测模型。
