Malaty Hoda M, Abraham Bincy P, Mehta Seema, Garnett Elizabeth A, Ferry George D
Department of Medicine, Houston, TX, USA ; Department of Pediatrics, Houston, TX, USA.
Clin Exp Gastroenterol. 2013 Jun 17;6:77-83. doi: 10.2147/CEG.S40259. Print 2013.
The natural history of ulcerative colitis (UC) has been poorly studied in children.
We performed a retrospective study in children diagnosed with UC with a follow-up. The diagnosis of UC was based on clinical, radiologic, endoscopic, and histologic examinations. We estimated the occurrence of colectomy, proctitis, and extraintestinal manifestations (EIMs) at the onset of the diagnosis and at the end of the study period.
We identified 115 UC patients between 1986 and 2003 with a mean age at diagnosis of 10.6 ± 5.1 years. The cumulative rate of colectomy was 4.1% at 1 year, and 16% at 10 years. EIMs were experienced by 20% of the children; 48% had arthritis, 35% had sclerosing cholangitis, and 17% had aphthous stomatitis. Proctitis was noted in 29 patients and it was not associated with an increased risk of colectomy (relative risk = 1.4; 95% CI = 0.7-4.5), and girls were twice more likely to develop proctitis. The pathologic reading for disease extensions was recorded for all children at entry and only 62 children had pathological results at maximum follow-up. At entry, 25% of the children only had ulcerative proctitis (E1) localization, 40% had left-sided UC (E2), and 35% had extensive UC (E3). Among the patients with E1 localization, 20% had progressed to E2 and 80% had progressed to E3; among the patients with E2 localization, 40% had progressed to E3. Age, gender, and EIMs at time of diagnosis were not associated with extension of disease at maximal follow-up. The Z score of body mass index (BMI) of children was significantly higher at the end of the study. At diagnosis, 85% of patients received 5-aminosalicyclic acid, 60% received steroids, and 11% received an immunomodulator. The majority of patients were still using systemic steroids at and after 5 years from their entry date. Only 32 of the 91 children on steroids did not receive an immunomodulator.
Pediatric UC is associated with high rates of EIMs and colectomy that are not dependent on age, gender, or race, but is associated with a high rate of proctitis among girls. Understanding the clinical course of UC can optimize therapeutic interventions.
儿童溃疡性结肠炎(UC)的自然病史研究较少。
我们对诊断为UC的儿童进行了一项随访回顾性研究。UC的诊断基于临床、放射学、内镜和组织学检查。我们评估了诊断开始时和研究期末结肠切除术、直肠炎及肠外表现(EIMs)的发生率。
我们确定了1986年至2003年间的115例UC患者,诊断时的平均年龄为10.6±5.1岁。结肠切除术的累积发生率在1年时为4.1%,10年时为16%。20%的儿童有EIMs;48%有关节炎,35%有硬化性胆管炎,17%有复发性口疮性口炎。29例患者有直肠炎,其与结肠切除术风险增加无关(相对风险=1.4;95%可信区间=0.7 - 4.5),且女孩患直肠炎的可能性是男孩的两倍。所有儿童在入组时记录疾病扩展的病理诊断结果,仅62例儿童在最大随访时有病理结果。入组时,25%的儿童仅为溃疡性直肠炎(E1)定位,40%为左侧UC(E2),35%为广泛性UC(E3)。在E1定位的患者中,20%进展为E2,80%进展为E3;在E2定位的患者中,40%进展为E3。诊断时的年龄、性别和EIMs与最大随访时疾病的扩展无关。研究结束时儿童体重指数(BMI)的Z评分显著更高。诊断时,85%的患者接受5 - 氨基水杨酸治疗,60%接受类固醇治疗,11%接受免疫调节剂治疗。大多数患者在入组日期后5年及5年后仍在使用全身性类固醇。在使用类固醇的91例儿童中,只有32例未接受免疫调节剂治疗。
儿童UC与高比例的EIMs和结肠切除术相关,这与年龄、性别或种族无关,但女孩患直肠炎的比例较高。了解UC的临床病程可优化治疗干预措施。