Thinking outside the box: a review of gastrointestinal symptoms and complications in cystic fibrosis

Gastrointestinal (GI)-related symptoms, complications, and comorbidities in cystic fibrosis (CF) are common and research to reduce their burden is a priority for the CF community. To enable future research, this review aimed to summarize the range of GI symptoms, complications and comorbidities seen in CF, the underlying pathophysiology, and treatments. This was a rapid systematic review undertaken using the recommendations from the Cochrane Rapid Reviews Methods Group. We searched databases including PubMed, Embase, Medline and the Cochrane database and identified those studies reporting GI-related symptoms, complications, or comorbidities in CF or their treatment. Our searches identified 2,930 studies and a total 119 studies met our inclusion criteria. Where a prevalence could be determined, GI symptoms were reported in 33.7% of study participants. The range of symptoms reported was broad and the highest median prevalence included flatulence (43.5%), bloating and abdominal distension (36%), and fatty stool (36%). Meconium ileus was reported in 12% and distal intestinal obstruction syndrome in 8.5% GI-related symptoms, complications, and comorbidities in CF are common. More consistent characterization and recording of these symptoms in clinical studies may help achieve the priority of reducing the burden of GI disease in CF.

囊性纤维化（cystic fibrosis, CF）患者的胃肠道（Gastrointestinal, GI）相关症状、并发症与合并症十分常见，减轻此类病症负担的相关研究是CF领域的重点研究方向。为支撑后续研究开展，本快速系统综述旨在梳理CF患者中各类胃肠道相关症状、并发症与合并症的表现谱，以及其潜在病理生理机制与治疗方案。本研究遵循Cochrane快速综述方法小组的指南，开展了此项快速系统综述。我们检索了包括PubMed、Embase、Medline及Cochrane数据库在内的多个文献库，筛选出报道CF患者胃肠道相关症状、并发症、合并症或其治疗方案的相关研究。本次检索共获取2930项研究，最终有119项研究符合纳入标准。在可明确患病率的研究中，有33.7%的研究对象报告存在胃肠道症状。本次报道的症状谱较为广泛，患病率中位数最高的症状包括胃肠胀气（43.5%）、腹胀与腹部膨隆（36%）以及脂肪泻（36%）。胎粪性肠梗阻（Meconium ileus）的报告患病率为12%，远端肠梗阻综合征（distal intestinal obstruction syndrome）为8.5%。CF患者的胃肠道相关症状、并发症与合并症十分常见。在临床研究中对这类症状进行更统一的特征描述与记录，将有助于实现减轻CF患者胃肠道疾病负担这一重点研究目标。