Zhang R, Zhong X M, Gong Y Z, Ma X, Zhu D, Ning H J, Wang F P, Zou J Z, Zhang Y L
Department of Gastroenterology, Children's Hospital, Capital Institute of Pediatrics, Beijing 100020, China.
Department of Pathology, Children's Hospital, Capital Institute of Pediatrics, Beijing 100020, China.
Zhonghua Er Ke Za Zhi. 2020 Jul 2;58(7):570-575. doi: 10.3760/cma.j.cn112140-20200504-00468.
To analyze the clinical characteristics and efficacy of drug treatment in children with inflammatory bowel disease (IBD) at different ages of onset. 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. (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 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 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, 0.003). Also, the location of bowel inflammation among the four groups were significantly different (χ(2)=21.120, 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 6(th) week of treatment, and the proportion of remission maintenance at the 30(th) 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. 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.
分析不同发病年龄儿童炎症性肠病（IBD）的临床特征及药物治疗疗效。收集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收治的87例IBD患儿的临床资料。根据发病年龄将患者分为四组：0～<2岁组（36例）、2～<6岁组（10例）、6～<10岁组（12例）和10～<18岁组（29例）。采用卡方检验或Fisher确切概率法比较不同年龄组的临床表现、实验室检查、内镜检查结果、病理及基因变化以及治疗情况。（1）87例患者确诊为IBD，其中克罗恩病（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）0～<2岁组和2～<6岁组发热患者分别占78%（28/36）和8/10。0～<2岁组腹痛和肛周疾病患者分别占6%（2/36）和47%（17/36），四组间比例差异有统计学意义（χ²=8.369、40.317和13.130，均P<0.05）。（3）0～<2岁组白细胞增多、血小板增多和贫血更为常见，分别占72%（26/36）、31%（11/36）和81%（29/36）。四组血常规变化差异有统计学意义（χ²=21.919、8.095和11.520，均P<0.05）。（4）0～<2岁的CD患儿结肠受累占85%（17/20）。而6岁以上的CD患儿中，61%（14/23）有回肠和结肠炎症，与6岁以下患儿相比差异有统计学意义（19%（5/27），χ²=9.455，P=0.003）。此外，四组肠道炎症部位差异有统计学意义（χ²=21.120，P=0.01）。（5）15例（30%）CD患者发现非干酪样肉芽肿，11例（44%）UC患者发现隐窝脓肿。（6）在24例经高通量测序分析基因的患者中，12例有致病性单基因突变。（7）25例患者接受了全肠内营养治疗。在25例接受沙利度胺治疗的患者中，20例（80%）临床缓解或部分缓解。在19例接受英夫利昔单抗（IFX）治疗的CD患者中，14例在治疗第6周临床缓解，治疗第30周缓解维持率为12/14。（8）0～<2岁组临床缓解或部分缓解率为64%（23/36），2～<6岁组为8/10，6～<10岁组为11/12，10～<18岁组为83%（24/29）。本研究中CD的比例高于UC。婴儿期起病的炎症性肠病更易出现肛周病变，通常伴有白细胞增多、血小板增多和贫血，且单基因突变可能性大。IFX可能对治疗CD有效。
