Renal involvement in tuberous sclerosis complex and von Hippel-Lindau disease: shared disease mechanisms?
Renal involvement in tuberous sclerosis complex and von Hippel-Lindau disease: shared disease mechanisms?
复制标题
结节性硬化症和 von Hippel-Lindau 病的肾脏受累：共同的疾病机制？
DOI：
10.1038/ncpneph1032
复制
发表时间：
2009
影响因子：
--
通讯作者：
Bissler,JohnJ
中科院分区：
文献类型：
--
作者：
Siroky,BrianJ;Czyzyk-Krzeska,MariaF;Bissler,JohnJ
结节性硬化症和冯希佩尔-林道病是不同的常染色体显性肿瘤抑制综合征，它们可以表现出相似的肾脏表型，并且似乎共享一些信号通路成分。相似之处存在于当前的临床管理和新发现的潜在治疗方法，这些条件。本综述总结了结节性硬化症和希佩尔-林道病之间的病理生理和治疗重叠，并强调了最近在这些情况下进行的药物试验的结果。
Tuberous sclerosis complex and von Hippel–Lindau disease are distinct autosomal dominant tumor suppressor syndromes that can exhibit similar renal phenotypes and seem to share some signaling pathway components. Similarities exist in the current clinical management of, and the newly identified potential therapeutic approaches for, these conditions. This Review summarizes the pathophysiologic and therapeutic overlap between tuberous sclerosis complex and von Hippel–Lindau disease and highlights the results of recent drug trials in these settings.