囊性纤维化婴儿的管理：囊性纤维化基金会工作组对囊性纤维化婴儿护理的证据总结。

Management of infants with cystic fibrosis: a summary of the evidence for the cystic fibrosis foundation working group on care of infants with cystic fibrosis.

机构信息

Department of Medicine, Johns Hopkins University, Baltimore, MD, USA.

出版信息

J Pediatr. 2009 Dec;155(6 Suppl):S94-S105. doi: 10.1016/j.jpeds.2009.09.002.

DOI:10.1016/j.jpeds.2009.09.002

PMID:19914446

Abstract

OBJECTIVE

To inform the development of Cystic Fibrosis (CF) Foundation guidelines on the care of infants with CF, we systematically reviewed the evidence for diagnosis and assessment of pancreatic and pulmonary disorders; management of pancreatic and pulmonary function; management of nutrition and nutritional disorders; and prevention and control of infections.

STUDY DESIGN

In May-June 2008, we searched The Cochrane Library for existing reviews; and MEDLINE, the National Guideline Clearinghouse, the CF Foundation Clinical Practice Guidelines and Consensus Statements, and the UK CF Trust for existing guidelines. MEDLINE, Cumulative Index to Nursing and Allied Health Literature (CINAHL) and the Excerpta Medica Database (EMBASE) were searched for primary studies in January 2008. Bibliographies of eligible articles were searched and expert input was sought. We selected English-language articles of any study design that provided original data on any of our questions on infants up to 2 years of age.

RESULTS

We identified 14 relevant guidelines and 3 Cochrane reviews. Fifty-nine articles (55 primary studies) were included. Only four of these were randomized controlled trials. Sample sizes of infants ranged from 2 to 768 study participants; the median sample size was 24. Of our 21 review topics, 5 topics had only one study while for 5 we identified no relevant studies. We identified one or no primary studies for 20 of 32 review questions.

CONCLUSIONS

There is a paucity of evidence on the care of infants diagnosed with CF. For several of the review questions no guidelines or primary studies were identified, but for other questions, studies limited by weak design and small sample sizes were the only studies identified. With increasing numbers of infants with CF being diagnosed by newborn screening there is an opportunity to study the management of infants diagnosed with CF.

摘要

目的

为了为囊性纤维化基金会（CF）制定关于囊性纤维化婴儿护理的指南提供信息，我们系统地回顾了有关胰腺和肺部疾病诊断和评估、胰腺和肺功能管理、营养和营养障碍管理以及感染预防和控制的证据。

研究设计

2008 年 5 月至 6 月，我们在 Cochrane 图书馆中搜索了现有的综述，并在 MEDLINE、国家指南清理中心、CF 基金会临床实践指南和共识声明以及英国 CF 信托基金中搜索了现有的指南。2008 年 1 月，我们在 MEDLINE、护理与联合健康文献累积索引（CINAHL）和 Excerpta Medica 数据库（EMBASE）中搜索了关于 2 岁以下婴儿的任何问题的原始研究。还搜索了合格文章的参考文献，并征求了专家的意见。我们选择了任何研究设计的英文文章，这些文章提供了关于我们提出的任何婴儿问题的原始数据。

结果

我们确定了 14 项相关指南和 3 项 Cochrane 综述。共纳入 59 篇文章（55 篇原始研究）。只有 4 项为随机对照试验。婴儿样本量范围为 2 至 768 名研究参与者；中位数样本量为 24。在我们的 21 个综述主题中，有 5 个主题只有一项研究，而对于 5 个主题，我们没有发现相关研究。对于 32 个综述问题中的 20 个，我们只确定了一个或没有原始研究。