时间标签：数据日期 2026-11-01 · 生成时间 2026-11-01 05:30（Asia/Shanghai）｜ 检索窗口 PubMed edat 2026/10/31–2026/11/01 ｜ 情报发生日期 2026-11-01
研究问题
The clinical course is presented in two panels (≤120 days and >120 days) to illustrate the early treatment response and long‐term follow‐up.；BR, bowel rest; EN, enteral nutrition; FC, fecal calprotectin; PN, parenteral nutrition; PO, oral intake; PUCAI, Pediatric Ulcerative Colitis Activity Index. 3
方法
Because of small intestinal obstruction, jejunal anastomosis and enterostomy were performed on day 1 of life, and the stoma was closed at 3 months of age.；Before initiating vedolizumab therapy, infectious disease screening was performed, including stool cultures, Clostridioides difficile toxin, HIV, hepatitis B virus, hepatitis C virus, Epstein–Barr virus, cytomegalovirus, and T‐SPOT.TB…
核心结论
Previous reports on RTEL1 ‐associated enterocolitis have documented its refractoriness to standard IBD treatments. , Patients with HHS exhibit combined immunodeficiency associated with telomere dysfunction, leading to increased susceptibility to infections.；In such patients, the use of systemic immunosuppressive therapies may be associated with a higher risk of severe infections, making therapeutic decisions particularly challenging.；CONCLUSION We report a child with HHS who developed enterocolitis and was successfully treated with vedolizumab without major adverse events.
局限
Further studies are needed to establish its long‐term efficacy and safety.
对本项目的价值
参考性证据：炎症衰老是多数慢病共病的上游枢纽，可用于分层与联合干预设计。 ｜ 价值分 1.2/5（★☆☆☆☆）
全文来源
PubMed PMC JATS 全文（正文 8,421 字符，已过 DOI 逐字 + 题名指纹双重归属校验）
原文题录
- 英文标题：Vedolizumab use for severe chronic enterocolitis in a child with Hoyeraal-Hreidarsson syndrome: A case report.
- 期刊与标识：JPGN reports · PMID 42959554 · DOI 10.1002/jpr3.70291
A 级证据分级：A 级（同行评议人体研究）
本文属人体研究证据，但需注意：观察性或小样本干预的结论不等于已证实临床获益；效应量与长期安全性仍需更大规模、更长随访的试验验证。
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