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极早发型炎症性肠病的临床病程。

Clinical Course of Very Early-Onset Inflammatory Bowel Disease.

机构信息

From the Pediatric Gastroenterology and Cystic Fibrosis Unit, Department of Human Pathology in Adulthood and Childhood "G. Barresi", University of Messina, Messina, Italy.

the Pediatric Gastroenterology and Endoscopy Unit, Institute "Giannina Gaslini", Genoa, Italy.

出版信息

J Pediatr Gastroenterol Nutr. 2023 May 1;76(5):590-595. doi: 10.1097/MPG.0000000000003730. Epub 2023 Feb 7.

DOI:10.1097/MPG.0000000000003730
PMID:36754082
Abstract

OBJECTIVES

Data on the phenotypes and disease outcomes of very early-onset inflammatory bowel disease (VEO-IBD) are limited. The aims of this study were to describe the clinical features, outcomes, and treatment response of VEO-IBD patients and to compare them with later-onset pediatric inflammatory bowel disease (P-IBD) patients.

METHODS

All consecutive patients aged 0-6 years who were diagnosed with Crohn disease (CD), ulcerative colitis, or IBD unclassified (IBD-U) at 2 academic hospitals from 2010 to March 2021 were included. They were compared to sex-matched IBD patients aged 6-17 years.

RESULTS

Two hundred thirty-two patients were included, 78 (34%) with VEO-IBD and 154 (66%) with P-IBD. IBD-U was the most common diagnosis in the VEO-IBD group compared to P-IBD (28% vs 3%, P < 0.001), while CD was predominant in older children (27% vs 52%, P < 0.001). The VEO-IBD group showed lower rates of clinical remission after induction with steroids compared to older children (82% vs 93%, P = 0.01), higher rates of steroid resistance (14% vs 5%, P = 0.02), and steroid dependence (27% vs 8%, P < 0.001). The number of patients who started anti-tumor necrosis factor (TNF)-α agents was similar between the groups. Anti-TNF-α retention was lower in the VEO-IBD group at 1 and 2 years (59% vs 85%, P = 0.003; 16% vs 55%, P < 0.001, respectively). Surgical risk appeared to be higher for VEO-IBD (32% vs 14%, P < 0.001).

CONCLUSIONS

When compared to P-IBD patients, patients with VEO-IBD may have a more severe disease course, a poorer response to steroids and anti-TNF-α agents, and require more frequent surgical procedures.

摘要

目的

关于极早发型炎症性肠病(VEO-IBD)表型和疾病结局的数据有限。本研究旨在描述 VEO-IBD 患者的临床特征、结局和治疗反应,并将其与晚发型儿科炎症性肠病(P-IBD)患者进行比较。

方法

本研究纳入了 2010 年至 2021 年 3 月期间在 2 所学术医院诊断为克罗恩病(CD)、溃疡性结肠炎或 IBD 未分类(IBD-U)的年龄为 0-6 岁的连续患者,并与年龄匹配的 IBD 患者(6-17 岁)进行比较。

结果

共纳入 232 例患者,其中 78 例(34%)为 VEO-IBD,154 例(66%)为 P-IBD。与 P-IBD 相比,VEO-IBD 组中 IBD-U 是最常见的诊断(28%比 3%,P<0.001),而在年龄较大的儿童中 CD 更为常见(27%比 52%,P<0.001)。与年龄较大的儿童相比,VEO-IBD 组患者在诱导性类固醇治疗后达到临床缓解的比例较低(82%比 93%,P=0.01),类固醇耐药率较高(14%比 5%,P=0.02),类固醇依赖率较高(27%比 8%,P<0.001)。两组患者开始使用抗肿瘤坏死因子(TNF)-α 药物的人数相似。VEO-IBD 组在 1 年和 2 年时抗 TNF-α 药物的保留率较低(59%比 85%,P=0.003;16%比 55%,P<0.001)。VEO-IBD 的手术风险似乎更高(32%比 14%,P<0.001)。

结论

与 P-IBD 患者相比,VEO-IBD 患者的疾病可能更严重,对类固醇和抗 TNF-α 药物的反应较差,需要更频繁的手术。

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