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囊性纤维化结直肠癌筛查共识推荐意见。

Cystic Fibrosis Colorectal Cancer Screening Consensus Recommendations.

机构信息

Perelman School of Medicine, University of Pennsylvania Philadelphia, Pennsylvania.

Division of Gastroenterology, Hepatology, and Nutrition, University of Minnesota, Minneapolis, Minnesota.

出版信息

Gastroenterology. 2018 Feb;154(3):736-745.e14. doi: 10.1053/j.gastro.2017.12.012. Epub 2017 Dec 29.

DOI:10.1053/j.gastro.2017.12.012
PMID:29289528
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9675422/
Abstract

BACKGROUND & AIMS: Improved therapy has substantially increased survival of persons with cystic fibrosis (CF). But the risk of colorectal cancer (CRC) in adults with CF is 5-10 times greater compared to the general population, and 25-30 times greater in CF patients after an organ transplantation. To address this risk, the CF Foundation convened a multi-stakeholder task force to develop CRC screening recommendations.

METHODS

The 18-member task force consisted of experts including pulmonologists, gastroenterologists, a social worker, nurse coordinator, surgeon, epidemiologist, statistician, CF adult, and a parent. The committee comprised 3 workgroups: Cancer Risk, Transplant, and Procedure and Preparation. A guidelines specialist at the CF Foundation conducted an evidence synthesis February-March 2016 based on PubMed literature searches. Task force members conducted additional independent searches. A total of 1159 articles were retrieved. After initial screening, the committee read 198 articles in full and analyzed 123 articles to develop recommendation statements. An independent decision analysis evaluating the benefits of screening relative to harms and resources required was conducted by the Department of Public Health at Erasmus Medical Center, Netherlands using the Microsimulation Screening Analysis model from the Cancer Innervation and Surveillance Modeling Network. The task force included recommendation statements in the final guideline only if they reached an 80% acceptance threshold.

RESULTS

The task force makes 10 CRC screening recommendations that emphasize shared, individualized decision-making and familiarity with CF-specific gastrointestinal challenges. We recommend colonoscopy as the preferred screening method, initiation of screening at age 40 years, 5-year re-screening and 3-year surveillance intervals (unless shorter interval is indicated by individual findings), and a CF-specific intensive bowel preparation. Organ transplant recipients with CF should initiate CRC screening at age 30 years within 2 years of the transplantation because of the additional risk for colon cancer associated with immunosuppression.

CONCLUSIONS

These recommendations aim to help CF adults, families, primary care physicians, gastroenterologists, and CF and transplantation centers address the issue of CRC screening. They differ from guidelines developed for the general population with respect to the recommended age of screening initiation, screening method, preparation, and the interval for repeat screening and surveillance.

摘要

背景与目的

改善疗法大大提高了囊性纤维化(CF)患者的存活率。但与普通人群相比,CF 成人的结直肠癌(CRC)风险高 5-10 倍,器官移植后 CF 患者的风险高 25-30 倍。为了应对这一风险,CF 基金会召集了一个多利益相关方工作组来制定 CRC 筛查建议。

方法

由 18 名成员组成的工作组包括专家,包括肺病专家、胃肠病专家、社会工作者、护士协调员、外科医生、流行病学家、统计学家、CF 成人和家长。该委员会由 3 个工作组组成:癌症风险、移植和程序与准备。CF 基金会的一名指南专家于 2016 年 2 月至 3 月根据 PubMed 文献检索进行了证据综合。工作组成员进行了额外的独立搜索。共检索到 1159 篇文章。初步筛选后,委员会阅读了 198 篇全文,并分析了 123 篇文章以制定推荐意见。荷兰伊拉斯谟医学中心公共卫生系使用癌症神经支配和监测建模网络中的 Microsimulation Screening Analysis 模型,对筛查相对于危害和所需资源的益处进行了独立的决策分析。只有当建议达到 80%的接受阈值时,工作组才会在最终指南中包含推荐意见。

结果

工作组提出了 10 项 CRC 筛查建议,强调了共同的、个体化的决策制定以及对 CF 特定胃肠道挑战的熟悉程度。我们建议结肠镜检查作为首选筛查方法,筛查起始年龄为 40 岁,5 年再次筛查,3 年监测间隔(除非个人发现需要更短的间隔),以及 CF 特定的强化肠道准备。由于免疫抑制与结肠癌相关的额外风险,CF 器官移植受者应在移植后 2 年内,年龄 30 岁时开始 CRC 筛查。

结论

这些建议旨在帮助 CF 成人、家属、初级保健医生、胃肠病专家以及 CF 和移植中心解决 CRC 筛查问题。与为普通人群制定的指南相比,它们在筛查起始年龄、筛查方法、准备以及重复筛查和监测的间隔方面有所不同。

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本文引用的文献

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Gastroenterology. 2018 Feb;154(3):556-567.e18. doi: 10.1053/j.gastro.2017.10.036. Epub 2017 Nov 2.
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Cancer risk among lung transplant recipients with cystic fibrosis.患有囊性纤维化的肺移植受者的癌症风险。
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管理老龄化囊性纤维化患者群体:挑战与优先事项。
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The Aging Patient with Cystic Fibrosis.患有囊性纤维化的老年患者
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Functional variants in the cystic fibrosis transmembrane conductance regulator (CFTR) gene are associated with increased risk of colorectal cancer.囊性纤维化跨膜传导调节因子(CFTR)基因中的功能变异与结直肠癌风险增加相关。
Hum Mol Genet. 2025 Mar 20;34(7):617-625. doi: 10.1093/hmg/ddaf007.
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