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皮茨-杰格斯综合征:对复发性肠异常的管理
Anju Verma1, Pujana Kanneganti2, Basant Kumar2
1Department of Paediatric Surgery, SGPGIMS, Lucknow, 226014, India. anju_v2006@rediffmail.com.
Pediatric surgery international
|June 2, 2024
概括
皮茨-杰格斯综合征 (PJS) 患者往往需要进行多次手术以获得内. 建议在进行广泛的肠切除时进行保守的管理和有限的多角切除术,以预防短肠综合征.
科学领域:
- 儿科手术 儿科手术
- 胃肠病学 胃肠病学
- 遗传学 是一个遗传学.
背景情况:
- 皮茨-杰格斯综合征 (PJS) 是一种自体主导性疾病.
- 它的特点是胃肠道的哈马托马型多体和粘膜皮肤的斑点.
- 由于多次手术,PJS中反复发生的肠可以导致短肠综合征.
研究的目的:
- 介绍儿童PJS患者的治疗经验,这些患者有复发性内肠.
- 在这些复杂的案件中解决管理困境.
- 评估不同手术和保守方法的结果.
主要方法:
- 儿童PJS患者的回顾性综述,这些患者有反复的静脉置 (2015年1月 - 2023年12月).
- 收集的数据,介绍,管理和后续行动.
- 基于世界卫生组织 (WHO) 标准的诊断.
主要成果:
- 九名患者 (4-17岁) 呈现了复发性肠置.
- 进行了18次腹腔切除术,11次在中心.
- 有限切除或带有多角切除的肠切除比广泛切除更受青 (8/11例).
结论:
- 患有PJS的儿童面临多次腹腔切除术的高风险,因为多.
- 由于肠道的扩散参与,应避免进行广泛的肠切除.
- 在存在外科手术困境时,偏好保守的管理和有限的多角切除术.
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