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囊性纤维化的胃肠道腔面表现。

Luminal Gastrointestinal Manifestations of Cystic Fibrosis.

机构信息

Division of Gastroenterology and Hepatology, Saint Louis University, St. Louis, MI, USA.

Department of Gastroenterology and Hepatology, University of Michigan, Ann Arbor, MI, USA.

出版信息

Curr Gastroenterol Rep. 2021 Mar 23;23(3):4. doi: 10.1007/s11894-021-00806-5.

DOI:10.1007/s11894-021-00806-5
PMID:33758994
Abstract

PURPOSE OF REVIEW

People with cystic fibrosis (CF) are living longer. General age-related and CF-specific gastrointestinal symptoms are increasingly recognized. In this article, we review the latest data on luminal gastrointestinal manifestations in CF.

RECENT FINDINGS

People with CF have increased incidence of gastroesophageal reflux disease symptoms and often prescribed proton-pump inhibitors (PPI). PPI use may increase risk of pulmonary exacerbations. Evidence to support gastric fundoplication to improve pulmonary outcomes is limited. Features of intestinal dysmotility are common. There are distinct differences in the gut microbiome in the CF population which may have clinical implications. CF is a possible hereditary digestive cancer syndrome, particularly in regard to colorectal cancer (CRC) with earlier incidence of CRC and advanced colonic neoplasia. Early screening colonoscopy is warranted in the CF population. Gastrointestinal manifestations in CF are prevalent across all digestive organs. More study on the effect of interventions for symptomatic treatment and cancer screening is needed.

摘要

目的综述

囊性纤维化 (CF) 患者的寿命越来越长。人们越来越认识到与年龄相关的一般和 CF 特异性胃肠道症状。本文综述了 CF 腔内胃肠道表现的最新数据。

最新发现

CF 患者胃食管反流病症状的发生率增加,经常开质子泵抑制剂 (PPI)。PPI 的使用可能会增加肺部恶化的风险。支持胃底折叠术改善肺部结果的证据有限。肠道动力障碍的特征很常见。CF 人群的肠道微生物组存在明显差异,这可能具有临床意义。CF 可能是一种遗传性消化癌综合征,特别是在结直肠癌 (CRC) 方面,CRC 的发病率更早,结肠高级别瘤变更常见。CF 人群需要进行早期筛查结肠镜检查。CF 患者的胃肠道表现普遍存在于所有消化器官中。需要更多的研究来干预症状治疗和癌症筛查。

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mBio. 2024 Feb 14;15(2):e0193523. doi: 10.1128/mbio.01935-23. Epub 2024 Jan 26.
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Elexacaftor-Tezacaftor-Ivacaftor Treatment Reduces Abdominal Symptoms in Cystic Fibrosis-Early results Obtained With the CF-Specific CFAbd-Score.

本文引用的文献

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Development of the gut microbiota in early life: The impact of cystic fibrosis and antibiotic treatment.早期生命中肠道微生物组的发育:囊性纤维化和抗生素治疗的影响。
J Cyst Fibros. 2020 Jul;19(4):553-561. doi: 10.1016/j.jcf.2020.04.007. Epub 2020 May 30.
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ACG Clinical Guideline: Small Intestinal Bacterial Overgrowth.ACG 临床指南:小肠细菌过度生长。
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Role of ion channels in gastrointestinal cancer.离子通道在胃肠道癌症中的作用。
依列卡福妥-替扎卡福妥-依伐卡托治疗可减轻囊性纤维化患者的腹部症状——采用特定于囊性纤维化的CFAbd评分获得的早期结果
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Understanding Cystic Fibrosis Comorbidities and Their Impact on Nutritional Management.了解囊性纤维化合并症及其对营养管理的影响。
Nutrients. 2022 Feb 28;14(5):1028. doi: 10.3390/nu14051028.
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Changing epidemiology of the respiratory bacteriology of patients with cystic fibrosis-data from the European cystic fibrosis society patient registry.囊性纤维化患者呼吸道细菌学的流行病学变化:来自欧洲囊性纤维化协会患者登记处的数据。
J Cyst Fibros. 2020 May;19(3):376-383. doi: 10.1016/j.jcf.2019.08.006. Epub 2019 Sep 3.
5
A high prevalence of chronic gastrointestinal symptoms in adults with cystic fibrosis is detected using tools already validated in other GI disorders.使用已在其他胃肠道疾病中验证过的工具,在成年囊性纤维化患者中检测到慢性胃肠道症状的高发率。
United European Gastroenterol J. 2019 Aug;7(7):881-888. doi: 10.1177/2050640619841545.
6
Cystic fibrosis transmembrane conductance regulator modulates enteric cholinergic activities and is abnormally expressed in the enteric ganglia of patients with slow transit constipation.囊性纤维化跨膜电导调节因子调节肠胆碱能活性,并在慢传输性便秘患者的肠神经节中异常表达。
J Gastroenterol. 2019 Nov;54(11):994-1006. doi: 10.1007/s00535-019-01610-9. Epub 2019 Aug 7.
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Cystic fibrosis transmembrane conductance regulator (CFTR) and autophagy: hereditary defects in cystic fibrosis gluten-mediated inhibition in celiac disease.囊性纤维化跨膜传导调节因子(CFTR)与自噬:囊性纤维化中的遗传性缺陷 乳糜泻中麸质介导的抑制作用。
Oncotarget. 2019 Jul 9;10(43):4492-4500. doi: 10.18632/oncotarget.27037.
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A non-randomised single centre cohort study, comparing standard and modified bowel preparations, in adults with cystic fibrosis requiring colonoscopy.一项比较标准和改良肠道准备的非随机单中心队列研究,纳入需要结肠镜检查的成年囊性纤维化患者。
BMC Gastroenterol. 2019 Jun 13;19(1):89. doi: 10.1186/s12876-019-0979-z.
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Cystic fibrosis is associated with an increased risk of Barrett's esophagus.囊性纤维化与巴雷特食管的风险增加有关。
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The role of small intestinal bacterial overgrowth in cystic fibrosis: a randomized case-controlled clinical trial with rifaximin.小肠细菌过度生长在囊性纤维化中的作用:利福昔明的随机病例对照临床试验。
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