Lee Way Seah, Ng Ruey Terng, Chan Koon-Wing, Lau Yu-Lung
Way Seah Lee, Ruey Terng Ng, Department of Paediatrics, University Malaya Medical Center, Kuala Lumpur 59100, Malaysia.
World J Gastroenterol. 2016 Dec 28;22(48):10653-10662. doi: 10.3748/wjg.v22.i48.10653.
Infantile-onset inflammatory bowel disease (IO-IBD) with the onset of disease before 12 mo of age, is a different disease entity from childhood IBD. We aimed to describe the clinical features, outcome and role of mutation in interleukin-10 (IL-10) and interleukin-10 receptors (IL-10R) in Asian children with IO-IBD.
All cases of IO-IBD, defined as onset of disease before 12 mo of age, seen at University Malaya Medical Center, Malaysia were reviewed. We performed mutational analysis for and genes in patients with presenting clinical features of Crohn's disease (CD).
Six [13%; CD = 3, ulcerative colitis (UC) = 2, IBD-unclassified (IBD-U) = 1] of the 48 children (CD = 25; UC = 23) with IBD have IO-IBD. At final review [median (range) duration of follow-up: 6.5 (3.0-20) years], three patients were in remission without immunosuppression [one each for post-colostomy (IBD-U), after standard immunosuppression (CD), and after total colectomy (UC)]. Three patients were on immunosuppression: one (UC) was in remission while two (both CD) had persistent disease. As compared with later-onset disease, IO-IBD were more likely to present with bloody diarrhea (100% 55%, = 0.039) but were similar in terms of an associated autoimmune liver disease (0% 19%, = 0.31), requiring biologics therapy (50% 36%, = 0.40), surgery (50% 29%, = 0.27), or achieving remission (50% 64%, = 0.40). No mutations in either IL10 or IL10R in the three patients with CD and the only patient with IBD-U were identified.
The clinical features of IO-IBD in this Asian cohort of children who were negative for or mutations were variable. As compared to childhood IBD with onset of disease after 12 mo of age, IO-IBD achieved remission at a similar rate.
婴儿期起病的炎症性肠病（IO-IBD）是指在12月龄前发病的疾病，与儿童期炎症性肠病是不同的疾病实体。我们旨在描述亚洲IO-IBD儿童的临床特征、结局以及白细胞介素-10（IL-10）和白细胞介素-10受体（IL-10R）突变的作用。
回顾了马来西亚马来亚大学医学中心诊治的所有IO-IBD病例，即发病年龄在12月龄前的病例。我们对具有克罗恩病（CD）临床表现的患者进行了IL10和IL10R基因的突变分析。
48例炎症性肠病患儿（CD = 25例；溃疡性结肠炎（UC） = 23例）中有6例（13%；CD = 3例，UC = 2例，未分类炎症性肠病（IBD-U） = 1例）为IO-IBD。在末次随访时[随访时间中位数（范围）：6.5（3.0 - 20）年]，3例患者在未使用免疫抑制剂的情况下处于缓解期[分别为结肠造口术后（IBD-U）1例、标准免疫抑制治疗后（CD）1例、全结肠切除术后（UC）1例]。3例患者在使用免疫抑制剂：1例（UC）处于缓解期，而2例（均为CD）病情持续。与晚发型疾病相比，IO-IBD更易出现血便（100%对55%，P = 0.039），但在合并自身免疫性肝病方面相似（0%对19%，P = 0.31），在需要生物制剂治疗（50%对36%，P = 0.40）、手术治疗（50%对29%，P = 0.27）或实现缓解方面（50%对64%，P = 0.40）也相似。在3例CD患者和唯一1例IBD-U患者中均未发现IL10或IL10R的突变。
在这一亚洲儿童队列中，IL10或IL10R突变阴性的IO-IBD临床特征各异。与12月龄后起病的儿童期炎症性肠病相比，IO-IBD实现缓解的比例相似。
