Henen Sara, Denton Christine, Teckman Jeff, Borowitz Drucy, Patel Dhiren
Saint Louis University School of Medicine, SSM Health Cardinal Glennon Children's Hospital, 1465 S Grand Blvd, St. Louis, MO 63104.
Saint Louis University School of Medicine, SSM Health Cardinal Glennon Children's Hospital, 1465 S Grand Blvd, St. Louis, MO 63104.
J Cyst Fibros. 2021 Jul;20(4):578-585. doi: 10.1016/j.jcf.2021.05.016. Epub 2021 Jun 17.
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，且最近已成为研究热点。尽管有许多推测，包括管腔pH值、细菌过度生长和微生物群异常等因素，但CF人群中胃肠道（GI）功能障碍仍未得到充分了解。然而，动力障碍正逐渐成为CF肠道症状的一个可能关键因素。我们的综述文章旨在探讨在动物和人类研究中，囊性纤维化跨膜传导调节因子（CFTR）基因缺陷对胃肠道的后遗症，描述CF中肠道动力障碍的各种表现，回顾包括腔内测压在内的更新的诊断动力技术，并回顾当前关于动力障碍在CF相关肠道病理中的潜在作用的文献。
