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婴幼儿期起病的炎症性肠病有多种长期预后情况。

Infantile-onset inflammatory bowel disease has variable long-term outcomes.

作者信息

Krauthammer Alex, Weintraub Ilana, Shaoul Ron, Lev-Tzion Raffi, Broide Efrat, Wilschanski Michael, Lerner Aaron, Yerushalmi Baruch, Shouval Dror S, Shamaly Hussein, Haberman-Ziv Yael, Weiss Batia

机构信息

Pediatric Gastroenterology Unit, Edmond and Lily Safra Children's Hospital, Sheba Medical Center, Tel hashomer, Israel.

Sackler Faculty of Medicine, Tel-Aviv University, Tel Aviv, Israel.

出版信息

Front Pediatr. 2023 Mar 1;11:1097779. doi: 10.3389/fped.2023.1097779. eCollection 2023.

Abstract

OBJECTIVE AND AIM

Infantile-onset inflammatory bowel disease (IO-IBD), defined as IBD diagnosed at age 2 years or younger, tends to be more severe and refractory to conventional treatment than IBD diagnosed at a later age. However, data about IO-IBD and its long-term follow up are limited. We thus aimed to evaluate the presentation and long-term outcomes of patients with IO-IBD in a retrospective multicenter study.

METHODS

Medical records of patients diagnosed with IO-IBD in eight medical centers during 2000-2017 with at least 1-year follow up were reviewed. Demographics and disease characteristics at diagnosis including age of onset, disease phenotype and location, surgeries, medical therapy, and comorbid conditions were recorded.

RESULTS

Twenty-three patients with IO-IBD (16 males, 70%) were identified and followed for a median (range) of 51.2 (26.0-110.3) months. The mean ages at presentation and at the last follow up were 14 ± 9.8 and 101 ± 77 months, respectively. Six (26%) patients needed ileostomy already at the time of diagnosis and 20 (87%) were treated with corticosteroids. During long-term follow up, remission was achieved in 16 (73%) patients; of whom, 3 (14%) were without medications and 7 (32%) were in remission with the use of 5-aminosalicylic acid only. One patient needed hemicolectomy and one developed a severe EBV related infection.

CONCLUSION

The majority of patients with IO-IBD achieved long-term remission, despite a severe disease presentation at diagnosis. Surgery rate however is high, mainly during the first months from diagnosis.

摘要

目的与目标

婴幼儿期起病的炎症性肠病(IO-IBD)定义为在2岁及以下诊断的炎症性肠病,与较晚诊断的炎症性肠病相比,往往病情更严重且对传统治疗更具难治性。然而,关于IO-IBD及其长期随访的数据有限。因此,我们旨在通过一项回顾性多中心研究评估IO-IBD患者的临床表现和长期结局。

方法

回顾了2000年至2017年期间在8个医疗中心诊断为IO-IBD且至少随访1年的患者的病历。记录了诊断时的人口统计学和疾病特征,包括发病年龄、疾病表型和部位、手术、药物治疗和合并症。

结果

确定了23例IO-IBD患者(16例男性,占70%),中位(范围)随访时间为51.2(26.0 - 110.3)个月。就诊时和最后一次随访时的平均年龄分别为14±9.8个月和101±77个月。6例(26%)患者在诊断时即需要行回肠造口术,20例(87%)接受了皮质类固醇治疗。在长期随访中,16例(73%)患者实现缓解;其中,3例(14%)未使用药物,7例(32%)仅使用5-氨基水杨酸维持缓解。1例患者需要行半结肠切除术,1例发生了严重的EBV相关感染。

结论

尽管大多数IO-IBD患者在诊断时病情严重,但多数患者实现了长期缓解。然而,手术率较高,主要在诊断后的最初几个月。

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