儿童克罗恩病10例生长发育状况及临床特征分析

Growth and developmental status and clinical characteristics of 10 children with Crohn's disease

目的探讨儿童克罗恩病(CD)的生长发育状况、临床特征、治疗方案及转归。方法选取2020年1月至2022年9月于中山市人民医院诊断为CD的10例患儿临床资料,男7例,女3例,年龄10~14(12.65±1.08)岁。回顾性分析包括一般资料(年龄、性别、体格发育、性发育状况)、临床表现、实验室检查、影像学检查、内镜检查、内镜下黏膜组织活检病理特征、临床分型、活动度评分、治疗方案及转归。结果10例患儿典型临床表现有体质量减轻[90%(9/10)]、腹痛[80%(8/10)]、腹泻[40%(4/10)]。非典型临床表现有:贫血[80%(8/10)]、口腔溃疡[60%(6/10)]、发热[50%(5/10)]、肛周病变[50%(5/10)]。小肠CT成像提示有不同程度肠壁增厚、强化、肠腔狭窄。肠镜特征有溃疡[100%(10/10)]、卵石样外观[40%(4/10)]、息肉[40%(4/10)]、肛瘘[30%(3/10)]、狭窄[20%(2/10)]。病变部位以回结肠型最多见[80%(8/10)],其次为上消化道与回结肠混合型[20%(2/10)]。表现以非狭窄非穿透多见[80%(8/10)],其次为狭窄非穿透[20%(2/10)];疾病活动度以儿童克罗恩活动指数(PCDAI)评分,中/重度活动期7例,其中5例合并肛周病变,以抗肿瘤坏死因子-α单克隆抗体(TNF-α)进行诱导缓解及维持缓解治疗,随访期间4例进入临床缓解期,临床表现、内镜及肛周病变、生长发育状况均有所改善;2例无合并肛周病变的以泼尼松诱导缓解+硫唑嘌呤维持缓解治疗,随访后期出现疾病复发,均升级为TNF-α再度诱导缓解和维持缓解治疗;轻度活动期3例,均以全肠内营养+硫唑嘌呤诱导缓解及维持缓解治疗,现处于临床缓解期,1例达到内镜下黏膜愈合。结论深入全面的认识儿童CD,以期早期识别、诊断CD,避免诊治延误,进而制定精准个性化的治疗方案,降低患儿生长发育障碍等并发症发生率,改善患儿的生存质量。

Objective To explore the growth and development status,clinical characteristics,treatment,and outcomes of children with Crohn's disease(CD).Methods The clinical data of children diagnosed with CD at Zhongshan City People's Hospital from January 2020 to September 2022 were retrospectively analyzed,including general data(age,gender,and physical and sexual developmental status),clinical manifestations,laboratory examinations,imaging performance,endoscopy,pathological features of endoscopic mucosal biopsy,clinical classifications,activity evaluation,therapeutic options,and outcomes.There were 7 boys and 3 girls;they were 10.4~14.3(12.65±1.08)years old.Results The typical clinical manifestations were weight loss[90%(9/10)],abdominal pain[80%(8/10)],and diarrhea[40%(4/10)].The atypical clinical manifestations were anemia[80%(8/10)],oral ulcers[60%(6/10)],fever[50%(5/10)],and perianal lesions[50%(5/10)].The colonoscopy features were ulcer[100%(10/10)],pebble-like appearance[40%(4/10)],polypus[40%(4/10)],anal fistula[30%(3/10)],and stenosis[20%(2/10)].The most common lesion site was ileocolon type[80%(8/10)],followed by the mixed upper gastrointestinal and ileocolon type[20%(2/10)].The most common manifestation was non-stenosis and non-penetration[80%(8/10)],followed by stenosis and non-penetration[20%(2/10)].The activity was evaluated by the pediatric Crohn's disease activity index(PCDAI).There were 7 cases in moderate/severe active stage;5 of which were complicated with perianal lesions;anti-tumor necrosis factor-αmonoclonal antibody(TNF-α)was used to induce remission and maintain remission treatment.During the follow-up period,4 cases entered the clinical remission stage,and their clinical manifestations,endoscopy and perianal lesions,and growth and development status were improved.Two cases without perianal lesions were treated with prednisone-induced remission+azathioprine to maintain remission;when the disease recurred in the late follow-up period,both patients were upgraded to TNF-αre-induced remission and maintenance remission.Three cases of mild activity were treated with total enteral nutrition+azathioprine for induced remission and maintenance remission;then they were in clinical remission;1 case achieved mucosal healing under endoscope.Conclusions In-depth and comprehensive understanding of pediatric Crohn's disease is expected to identify and diagnose CD early,to avoid delay in diagnosis and treatment,and to develop accurate and personalized treatment programs to reduce the incidence of complications such as growth and development disorders in children and improve their quality of life.