同时的威尔姆斯瘤和自身主导的多囊性病
Andrew M Fleming1,2, Daniel B Gehle1,2, Melissa R Perrino3
1Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
Pediatric blood & cancer
|August 1, 2024
概括
威尔姆斯瘤 (WT) 和自体主导多囊性病 (ADPKD) 很少一起发生. 管理需要仔细考虑功能,特别是在切除术后,因为ADPKD可能在以后的生活中表现出来.
科学领域:
- 儿科瘤学 儿科瘤学
- 腎臟病學 (nephrology) 是一種醫學.
- 医学遗传学 医学遗传学
背景情况:
- 同时的威尔姆斯瘤 (WT) 和自身主导多囊性病 (ADPKD) 是非常罕见的.
- 同时诊断和管理对于儿科外科瘤学家来说构成重大挑战.
- 现有的文献对这些疾病的综合治疗和治疗提供了有限的指导.
研究的目的:
- 描述同时出现WT和ADPKD的患者的临床表现和管理.
- 审查WT和ADPKD的基础生物学和管理原则.
- 突出外科手术方法和术后护理方面的挑战.
主要方法:
- 在同一机构治疗的同时出现WT和ADPKD的患者的回顾性分析.
- 关于WT和ADPKD的生物学和管理的文献综述.
- 癌症倾向综合征的生殖系遗传测试.
主要成果:
- 介绍了三种单边WT和ADPKD治疗切除术的不同病例.
- ADPKD的诊断有所不同,有些人在手术前,手术期间或术后得到确认.
- 所有患者都存活了下来,但有一个人患上了末期功能衰竭,需要透析和移植.
结论:
- 同时出现的ADPKD和WT是罕见的;ADPKD的表现可能会延迟.
- ADPKD不是WT发展的已知风险因素.
- 在手术前对囊性病变进行表征,并讨论手术策略,因为切术后功能衰竭的风险至关重要.
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