单中心炎症性肠病患儿的临床特征及药物治疗分析

Analysis of clinical characteristics and drug treatment of inflammatory bowel disease in children:a single center study

目的探讨不同发病年龄儿童炎症性肠病(IBD)的临床特征,总结儿童IBD的临床特点和诊疗经验。方法回顾性分析2009年1月至2018年12月首都儿科研究所附属儿童医院消化内科确诊的87例IBD患儿的临床资料。按发病年龄分为4组,0~<2岁组(36例),2~<6岁组(10例),6~<10岁组(12例)及10~<18岁组(29例),比较不同年龄组患儿的临床表现、实验室检查、内镜下表现、病理、基因、治疗及预后。组间比较采用χ^2检验或Fisher精确分析。结果(1)87例IBD患儿中克罗恩病(CD)50例(57%),溃疡性结肠炎(UC)25例(29%),未分类炎症性肠病(IBD-U)12例(14%)。(2)发热在0~<2岁组及2~<6岁组分别占78%(28/36)、8/10;腹痛、肛周病变在0~<2岁组中占6%(2/36)及47%(17/36),3种症状在各年龄组患儿中差异有统计学意义(χ^2=8.369、40.317、13.130,P均<0.05)。(3)白细胞升高、血小板升高、血红蛋白减低在0~<2岁组中更多见,分别占72%(26/36)、31%(11/36)及81%(29/36),各年龄组患儿差异有统计学意义(χ^2=21.919、8.095、11.520,P均<0.05)。(4)0~<2岁组CD患儿病变以累及结肠为主,占85%(17/20),4年龄组患儿病变位置比较差异有统计学意义(χ^2=21.120,P<0.01)。≥6岁的CD患儿病变以累及回结肠为主,与<6岁CD组比较差异有统计学意义[61%(14/23)比19%(5/27),χ^2=9.455,P=0.003]。(5)15例(15/50,30%)CD患儿可见非干酪样肉芽肿,11例(11/25,44%)UC患儿可见隐窝脓肿。(6)行高通量基因测序的24例患儿中有12例为单基因突变。(7)25例患儿应用全肠内营养。25例患儿应用沙利度胺治疗,其中20例(80%)临床缓解或部分缓解。19例CD患儿应用英夫利昔单抗治疗,其中14例在应用IFX第6周时临床缓解,应用IFX第30周的维持缓解比例为12/14。(8)0~<2岁组、2~<6岁组、6~<10岁组、10~<18岁组患儿治疗后临床缓解或部分缓解的比例分别为64%(23/36)、8/10、11/12、83%(24/29)。结论本中心CD发病比例高于UC。婴幼儿IBD肛周病变发生率较高,且通常合并白细胞、血小板升高及贫血。婴幼儿IBD单基因突变可能性大。应用IFX治疗CD临床疗效明确。

Objective To analyze the clinical characteristics and efficacy of drug treatment in children with inflammatory bowel disease(IBD)at different ages of onset.Methods The clinical data of 87 children with IBD admitted to Department of Gastroenterology in Children′s Hospital,Capital Institute of Pediatrics from January 2009 to December 2018 were collected.The patients were divided into four groups according to the age of onset:0-<2 years old group(36 cases),2-<6 years old group(10 cases),6-<10 years old group(12 cases)and 10-<18 years old group(29 cases).The clinical manifestations,laboratory examination,endoscopic findings,pathologic and genetic changes,and treatment were compared among different age groups with chi-square test or Fisher′s exact text.Results(1)A total of 87 patients were diagnosed with IBD,including 50 Crohn′s disease(CD)(57%),25 ulcerative colitis(UC)(29%)and 12 unclassified inflammatory bowel disease(IBD-U)(14%).(2)Patients with fever accounted for 78%(28/36)and 8/10 in the 0-<2 years old group and 2-<6 years old group,respectively.Patients with abdominal pain and perianal diseases accounted for 6%(2/36)and 47%(17/36)in the 0-<2 years old group,and their proportions were significantly different among the four groups(χ^2=8.369,40.317 and 13.130,all P<0.05).(3)Leukocytosis,thrombocytosis and anemia were more common in the 0-<2 years old group,seen in 72%(26/36),31%(11/36)and 81%(29/36),respectively.There were significant differences in the changes of complete blood count among the four groups(χ^2=21.919,8.095 and 11.520,all P<0.05).(4)Colonic involvement accounted for 85%(17/20)in the 0-<2 years old CD patients.While in the CD patients over 6 years old,61%(14/23)had inflammation of ileum and colon,with a significant difference compared to that in patients under 6 years old(19%(5/27),χ^2=9.455,P=0.003).Also,the location of bowel inflammation among the four groups were significantly different(χ^2=21.120,P<0.01).(5)Noncaseating granulomas were found in 15(30%)CD patients,and crypt abscess was found in 11(44%)UC patients.(6)Among the 24 patients whose genes were analyzed by high throughput sequencing,12 had pathogenic single gene mutation.(7)There were 25 patients treated with total enteral nutrition.Among the 25 patients treated with thalidomide,20(80%)had clinical remission or partial remission.Among the 19 CD patients treated with infliximab(IFX),14 had clinical remission at the 6th week of treatment,and the proportion of remission maintenance at the 30th week of treatment was 12/14.(8)The rate of clinical remission or partial remission was 64%(23/36)in the 0-<2 years old group,8/10 in the 2-<6 years old group,11/12 in the 6-<10 years old group,and 83%(24/29)in the 10-<18 years old group.Conclusions The proportion of CD was higher than that of UC in this study.Infant onset inflammatory bowel disease was more likely to present with perianal lesions,and was usually associated with leukocytosis,thrombocytosis and anemia,and has high possibility of single gene mutation.IFX may be effective in treating CD.