Sathe Meghana Nitin, Freeman Alvin Jay
Division of Pediatric Gastroenterology and Nutrition, Children's Health, University of Texas Southwestern, F4.06, 1935 Medical District Drive, Dallas, TX 75235, USA.
Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta, Emory University, 2015 Uppergate Drive, Northeast, Atlanta, GA 30322, USA.
Pediatr Clin North Am. 2016 Aug;63(4):679-98. doi: 10.1016/j.pcl.2016.04.008.
Pulmonary disease is the primary cause of morbidity and mortality in people with cystic fibrosis (CF), but significant involvement within gastrointestinal, pancreatic, and hepatobiliary systems occurs as well. As in the airways, defects in CFTR alter epithelial surface fluid, mucus viscosity, and pH, increasing risk of stasis through the various hollow epithelial-lined structures of the gastrointestinal tract. This exerts secondary influences that are responsible for most gastrointestinal, pancreatic, and hepatobiliary manifestations of CF. Understanding these gastrointestinal morbidities of CF is essential in understanding and treating CF as a multisystem disease process and improving overall patient care.
肺部疾病是囊性纤维化（CF）患者发病和死亡的主要原因，但胃肠道、胰腺和肝胆系统也会受到显著影响。与气道情况一样，CFTR缺陷会改变上皮表面液体、黏液黏度和pH值，增加胃肠道各种中空上皮内衬结构发生淤滞的风险。这会产生继发性影响，导致CF的大多数胃肠道、胰腺和肝胆表现。了解CF的这些胃肠道病变对于将CF作为一种多系统疾病过程进行理解和治疗以及改善患者的整体护理至关重要。
