在家族性腺多重症中对德斯莫伊德病的管理
Joshua Sommovilla1,2, Dale Shepard3, David Liska1,2
1Department of Colon and Rectal Surgery, Digestive Disease and Surgery Institute, Cleveland Clinic, Cleveland, Ohio.
Clinics in colon and rectal surgery
|April 12, 2024
概括
德斯莫伊德病对患有家族性腺瘤多重症 (FAP) 的患者产生重大影响,导致严重的健康问题. 对这些良性瘤的管理策略是个性化的,从医疗疗法到新出现的肠移植.
科学领域:
- 在瘤学瘤学.
- 胃肠病学 胃肠病学
- 遗传学 是一个遗传学.
背景情况:
- 德斯莫伊德病是家族性腺瘤多重症 (FAP) 患者罹患和死亡的重要原因.
- 大约30%的FAP患者会发展出desmoid瘤,其严重程度差异很大.
- 风险因素包括女性性别,家族史,3'APC突变和肠外FAP表现.
研究的目的:
- 审查FAP患者的desmoid疾病的临床表现,风险因素和管理策略.
- 为突出现有和新兴的治疗选择的desmoid瘤.
主要方法:
- 在FAP中对desmoid疾病的文献综述.
- 分析已确定的风险因素和临床表现.
- 目前医疗和手术治疗方式的概述.
主要成果:
- 瘤具有广泛的严重程度,从无症状到危及生命.
- 治疗是根据瘤阶段,严重程度和患者解剖学的个性化.
- 医疗选择包括NSAID,激素治疗,TKI和细胞毒剂.
- 手术干预通常是有限的,可以考虑息选择和肠道移植.
结论:
- 在FAP中进行的Desmoid疾病管理需要一个量身定制的方法.
- 肠子移植为严重病例提供了一个有希望的,尽管数据有限的选择.
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