埃尔尼卡关于全结肠和肠道角膜炎的临床共识声明
Anna Löf Granström1, Willemijn Irvine2, Anders Telle Hoel3
1Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden; Unit of Pediatric Surgery, Karolinska University Hospital, Stockholm, Sweden.
这项研究开发了对影响整个结肠或肠道的罕见,危及生命的赫斯普朗格病变体的临床共识陈述. 专家建议重点关注这些复杂的儿科病例的手术治疗,肠道功能和长期护理.
科学领域:
- 儿科手术 儿科手术
- 胃肠病学 胃肠病学
- 罕见疾病 罕见疾病
背景情况:
- 赫施普隆病是一种由于缺少肠道质细胞而导致的先天性运动障碍.
- 总结肠或肠道结肠病是严重的变体,影响患者的比例低于10%.
- 这些情况带来了严重的危及生命的挑战,需要专门的管理.
研究的目的:
- 为ERNICA (欧洲罕见先天性消化系统疾病参考网络) 建立临床共识陈述.
- 为了解决全结肠和肠道结肠病的关键方面:
- 手术治疗,肠道功能不佳的管理和长期护理.
主要方法:
- 来自ERNICA中心的多学科小组参加了会议.
- 在Medline,Embase和Google学者中进行文献搜索.
- 一个经过修改的德尔菲进程在三轮中精炼了45个共识陈述.
主要成果:
- 来自10个欧洲国家的24名专家在45个声明上达成共识.
- 高质量的临床证据有限,许多陈述基于专家意见.
- 25个声明没有达成共识.
结论:
- 总结肠和肠道结肠病是罕见的希希斯普朗格病变体,证据有限.
- 该共识提供了关于手术治疗,肠功能和长期管理的指导.
- 建议在专家中心提供多学科,个性化的护理,以获得最佳的患者结果.
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