先天性巨结肠病和炎症性肠病患者的临床特征和诊断验证。
Clinical characteristics and validation of diagnosis in individuals with Hirschsprung disease and inflammatory bowel disease.
机构信息
Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Department of Surgery, Danderyd Hospital & Department of Clinical Sciences, Danderyd Hospital, Karolinska Institutet, Stockholm, SE, Sweden.
Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden; Department of Pediatrics, Örebro University Hospital, Örebro University, Örebro, Sweden; Division of Epidemiology and Public Health, School of Medicine, University of Nottingham, Nottingham, UK; Department of Medicine, Celiac Disease Center, Columbia University College of Physicians and Surgeons, New York, NY, USA.
出版信息
J Pediatr Surg. 2021 Oct;56(10):1799-1802. doi: 10.1016/j.jpedsurg.2020.11.015. Epub 2020 Nov 19.
BACKGROUND/PURPOSE: Hirschsprung disease has previously been reported to be associated with inflammatory bowel disease (IBD). The aim was to clinically confirm the diagnosis and to describe characteristics among individuals with both conditions in a national populationbased cohort.
METHODS
Nationwide, population-based cohort study, including all individuals with a Hirschsprung disease diagnosis and an IBD diagnosis registered between 1964 and 2016, in which clinical data were collected from the medical records of 18 validated cases with both Hirschsprung disease and IBD. The medical record of each individual in the study cohort was reviewed for age at IBD diagnosis, type of aganglionosis, type of surgical treatment, subtype of IBD, and treatment for IBD.
RESULTS
Median age at follow up was 34 years (range 19-66), and 3 of 18 indivduals (17%) were females. Median age at first diagnosis of IBD was 21 years (range 10-46). Six patients had ulcerative colitis, ten had Crohn's disease and two had unclassified IBD. Most of the patients had pharmacological treatment for IBD and 5 (28%) individuals had surgical treatment.
CONCLUSION
Hirschsprung disease and IBD was clinically confirmed in 18 cases. Age at IBD onset and subtype of IBD is similar to IBD patients without Hirschsprung disease. Five individuals had undergone surgical treatment for IBD.
背景/目的：先天性巨结肠症先前曾被报道与炎症性肠病（IBD）有关。本研究旨在通过全国性基于人群的队列研究来临床确诊该病，并描述具有这两种疾病的个体的特征。
方法
这是一项全国性基于人群的队列研究，纳入了所有在 1964 年至 2016 年间被诊断为先天性巨结肠症和 IBD 的个体。研究中，通过对 18 例明确患有先天性巨结肠症和 IBD 的患者的病历进行回顾，收集了临床数据。对研究队列中每位患者的病历进行了 IBD 诊断年龄、无神经节细胞病变类型、手术治疗类型、IBD 亚型和 IBD 治疗方式的回顾。
结果
中位随访年龄为 34 岁（范围 19-66 岁），3 名患者（17%）为女性。IBD 首次诊断的中位年龄为 21 岁（范围 10-46 岁）。6 例患者患有溃疡性结肠炎，10 例患者患有克罗恩病，2 例患者患有未分类的 IBD。大多数患者接受了 IBD 的药物治疗，5 名患者（28%）接受了手术治疗。
结论
18 例患者临床确诊患有先天性巨结肠症和 IBD。IBD 发病年龄和 IBD 亚型与无先天性巨结肠症的 IBD 患者相似。5 名患者接受了 IBD 的手术治疗。
Zotero 插件