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