Clinical features and outcomes of perianal disease in pediatric onset Crohn's disease.

IF 3.8 3区 医学 Q1 GASTROENTEROLOGY & HEPATOLOGY
Lin Wang, Yuhuan Wang, Ping Li, Shengnan Wang, Cuifang Zheng, Ying Huang
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引用次数: 0

Abstract

Background: Limited data have explored perianal involvement in pediatric Crohn's disease (CD). We aimed to investigate the characteristics and clinical outcomes of pediatric onset CD with perianal disease.

Methods: The medical records of diagnosed CD with perianal disease were reviewed retrospectively. The clinical characteristics, genetic testing, treatments and surgical outcomes among very early onset (VEO group) and later onset (LO group) were investigated. The occurrence of perianal fistulizing CD (pfCD) and intestinal resection were analyzed.

Results: Of the 155 included patients, 52.3 % and 47.7 % were enrolled in VEO and LO group, respectively. At diagnosis, VEO patients had more colonic and rectal involvement and higher disease activity score (P < 0.001). A monogenic disease was found in 94.9 % (74/78) of patients in VEO group and in 28.6 % (2/7) of patients in LO group. Infliximab treatment was more common among LO group (P < 0.001). VEO group had a higher rate of intestinal resection (P < 0.001). During follow-up, an additional 13 VEO patients and 8 LO patients developed pfCD. Moreover, B2 behavior and recto-vaginal fistulae were found to be predictive factors for intestinal resection.

Conclusion: VEOCD with perianal involvement is associated with higher monogenic mutation, more severe disease phenotypes, less utilization of biologics and higher rate of intestinal resection than LOCD.

小儿发病克罗恩病肛周疾病的临床特点和预后。
背景:关于儿童克罗恩病(CD)累及肛周的研究数据有限。我们的目的是探讨儿童发病的乳糜泻伴肛周疾病的特点和临床结果。方法:对诊断为乳糜泻伴肛周疾病的病例进行回顾性分析。观察极早发病组(VEO组)和晚发病组(LO组)的临床特点、基因检测、治疗及手术效果。对肛周瘘的发生情况及肠切除术进行分析。结果:155例纳入的患者中,VEO组和LO组分别占52.3%和47.7%。在诊断时,VEO患者有更多的结肠和直肠受累和更高的疾病活动评分(P < 0.001)。VEO组94.9%(74/78)的患者存在单基因疾病,LO组28.6%(2/7)的患者存在单基因疾病。英夫利昔单抗治疗在LO组中更为常见(P < 0.001)。VEO组肠切除率较高(P < 0.001)。随访期间,又有13例VEO患者和8例LO患者发生pfCD。此外,B2行为和直肠阴道瘘被发现是肠切除术的预测因素。结论:与lod相比,VEOCD伴肛周病变单基因突变率高、疾病表型严重、生物制剂利用率低、肠切除率高。
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来源期刊
Digestive and Liver Disease
Digestive and Liver Disease 医学-胃肠肝病学
CiteScore
6.10
自引率
2.20%
发文量
632
审稿时长
19 days
期刊介绍: Digestive and Liver Disease is an international journal of Gastroenterology and Hepatology. It is the official journal of Italian Association for the Study of the Liver (AISF); Italian Association for the Study of the Pancreas (AISP); Italian Association for Digestive Endoscopy (SIED); Italian Association for Hospital Gastroenterologists and Digestive Endoscopists (AIGO); Italian Society of Gastroenterology (SIGE); Italian Society of Pediatric Gastroenterology and Hepatology (SIGENP) and Italian Group for the Study of Inflammatory Bowel Disease (IG-IBD). Digestive and Liver Disease publishes papers on basic and clinical research in the field of gastroenterology and hepatology. Contributions consist of: Original Papers Correspondence to the Editor Editorials, Reviews and Special Articles Progress Reports Image of the Month Congress Proceedings Symposia and Mini-symposia.
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