Digestive System Research Unit, University Hospital Vall d'Hebron; Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (Ciberehd), Barcelona, Spain.
Department of Medicine, Universitat Autònoma de Barcelona, Barcelona, Spain.
Neurogastroenterol Motil. 2020 Sep;32(9):e13883. doi: 10.1111/nmo.13883. Epub 2020 May 31.
Cystic fibrosis (CF) is a multisystem disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Cystic fibrosis transmembrane conductance regulator is extensively expressed in the intestine and has an important role in the regulation of the viscosity and pH of gut secretions. Several studies have reported a delay in small bowel and colonic transit times in patients with CF which have been attributed to the secretory dysfunction. Our aim was to determine whether intestinal contractility is affected in these patients.
Consecutive patients with CF referred to our institution between 2014 and 2017 (n = 16) were prospectively investigated using automated non-invasive techniques for morpho-functional evaluation of the gut developed in our laboratory. On separate days, intraluminal images of the gut were obtained by capsule endoscopy and external images by abdominal MRI. Analysis of images (endoluminal and external) was performed with original, previously validated programs based on computer vision and machine learning techniques and compared with age- and sex-matched controls.
Patients with CF exhibited important reduction in contractile activity and increased retention of static turbid luminal content in the small bowel by endoluminal image analysis. Morpho-volumetric analysis of MRI images found increased ileo-colonic volumes in CF. Significant correlations between abnormalities detected by intraluminal and external imaging techniques were found. The presence and severity of digestive symptoms were not related to abnormal gut function.
Impaired transit and pooling of gut contents in patients with CF is associated with impaired intestinal motility.
囊性纤维化（CF）是一种多系统疾病，由囊性纤维化跨膜电导调节因子（CFTR）蛋白突变引起。囊性纤维化跨膜电导调节因子广泛表达于肠道，在调节肠道分泌物的黏度和 pH 值方面具有重要作用。几项研究报告称，CF 患者的小肠和结肠通过时间延迟，这归因于分泌功能障碍。我们的目的是确定这些患者的肠道收缩功能是否受到影响。
我们对 2014 年至 2017 年间我院收治的连续 CF 患者（n=16）进行前瞻性研究，采用我们实验室开发的自动非侵入性技术对肠道的形态和功能进行评估。在不同的日子里，通过胶囊内镜获取肠道腔内图像，通过腹部 MRI 获取外部图像。对图像（腔内和外部）进行分析，使用基于计算机视觉和机器学习技术的原始、先前验证的程序，并与年龄和性别匹配的对照组进行比较。
通过腔内图像分析，CF 患者的收缩活动明显减少，小肠中静态混浊腔内内容物的滞留增加。MRI 图像的形态容积分析发现 CF 患者的回结肠容积增加。腔内和外部成像技术检测到的异常之间存在显著相关性。消化症状的存在和严重程度与肠道功能异常无关。
CF 患者的肠道内容物通过和积聚不良与肠道运动功能障碍有关。
