Gastrointestinal manifestations in patients with cystic fibrosis (CF) are extremely common and have recently become a research focus. Gastrointestinal (GI) dysfunction is poorly understood in the CF population, despite many speculations including the role of luminal pH, bacterial overgrowth, and abnormal microbiome. Nevertheless, dysmotility is emerging as a possible key player in CF intestinal symptoms. Our review article aims to explore the sequelae of defective cystic fibrosis transmembrane conductance regulator (CFTR) genes on the GI tract as studied in both animals and humans, describe various presentations of intestinal dysmotility in CF, review newer diagnostic motility techniques including intraluminal manometry, and review the current literature regarding the potential role of dysmotility in CF-related intestinal pathologies.

囊性纤维化 (CF) 患者的胃肠道表现极为常见，最近已成为研究热点。尽管许多推测包括管腔 pH 值、细菌过度生长和异常微生物组的作用，但对 CF 人群的胃肠道 (GI) 功能障碍知之甚少。然而，运动障碍正在成为 CF 肠道症状的一个可能的关键因素。我们的评论文章旨在探索在动物和人类中研究的胃肠道缺陷性囊性纤维化跨膜电导调节器 (CFTR) 基因的后遗症，描述 CF 中肠道动力障碍的各种表现，回顾更新的诊断动力技术，包括腔内测压，以及回顾当前关于运动障碍在 CF 相关肠道病变中的潜在作用的文献。