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Abstract：
Introduction Previous studies have demonstrated an increased incidence of gastrointestinal (GI) pathologies, specifically celiac disease (CD) and eosinophilic esophagitis (EoE), in patients with cystic fibrosis (CF). However, there is minimal data available regarding endoscopic findings in pediatric patients with CF and GI mucosal disease. Methods A retrospective chart review was performed on patients with CF under 18 years of age who underwent esophagogastroduodenoscopy (EGD) or colonoscopy with biopsy over a 15-year period at our institution. Patient characteristics including assigned sex at birth, CF genetic mutations (if identified), and cystic fibrosis transmembrane conductance regulator (CFTR) modulator use were recorded. Data obtained at the time of biopsy included body mass index (BMI), indication for the procedure, exocrine pancreatic status, visual endoscopic findings, and histologic findings. Results A total of 72 patients with CF were included in the study. 24% (n=17) were found to have abnormal endoscopic biopsy results. EoE (13% of all patients, n=9) and CD (6% of all patients, n=4) were the most common GI diagnoses present on endoscopic biopsy. All 3 patients taking CFTR modulator medications at the time of endoscopy had normal biopsy results. Of the 17 patients found to have abnormal pathology results, 14 (82%) were taking proton-pump inhibitor (PPI) medication at the time of endoscopy. Conclusion This study highlights the probable increased frequency of GI disease in the pediatric CF population. These findings underscore the importance of maintaining a broad differential diagnosis while considering utilization of endoscopy with biopsy in pediatric patients with CF who have GI symptoms.
摘要：
介绍以前的研究表明，胃肠道（GI）病变的发生率增加，特别是乳糜泻（CD）和嗜酸性粒细胞性食管炎（EoE），囊性纤维化（CF）患者。然而,关于患有CF和GI粘膜疾病的儿科患者的内镜检查结果的数据很少.方法回顾性分析18岁以下CF患者在我们机构接受了15年的食管胃十二指肠镜（EGD）或结肠镜活检。患者特征包括出生时分配的性别，CF基因突变（如果确定），记录了囊性纤维化跨膜传导调节因子（CFTR）的使用情况。活检时获得的数据包括体重指数（BMI），该程序的指示，胰腺外分泌状态，视觉内窥镜检查结果，和组织学发现。结果共纳入72例CF患者。24％（n=17）被发现有异常的内镜活检结果。EoE（所有患者的13％，n=9）和CD（所有患者的6％，n=4）是内窥镜活检中最常见的胃肠道诊断。在内窥镜检查时服用CFTR调节剂药物的所有3名患者的活检结果均正常。在发现有异常病理结果的17例患者中，14人（82%）在内窥镜检查时服用质子泵抑制剂（PPI）药物。结论这项研究强调了小儿CF人群中胃肠道疾病的频率可能增加。这些发现强调了在有胃肠道症状的CF小儿患者中考虑采用内镜活检的同时保持广泛鉴别诊断的重要性。
