自體顯性多囊性腎病變之血管相關併發症

10.6666/ClinMed.201809_82(3).0100

李宗倫；林志慶

自體顯性多囊性腎病變(polycystic kidney disease) ； 顱內動脈瘤(intracranial aneurysm) ； 核磁共振影像

臨床醫學月刊

82卷3期（2018 / 09 / 28）

550 - 553

繁體中文

自體顯性多囊性腎病變(autosomal dominant polycystic kidney disease, ADPKD)為最常見的遺傳性腎臟疾病。腎臟內水泡的持續生長導致腎臟體積增大，日積月累導致腎臟衰竭。自體顯性多囊性腎病變病患可能同時存在血管相關之併發疾病，比如顱內動脈瘤可在近一成之無症狀之患者身上意外發現。自體顯性多囊性腎病變可依基因PKD1與PKD2作為分類。二者皆有因基因突變導致平滑肌細胞內鈣離子傳導變化，也因而導致血管構造改變容易發生動脈瘤。顱動脈瘤之剝離或破裂之發生更加重不良預後以及死亡。其他血管瘤例如胸主動脈瘤，腹腔主動脈瘤，主動脈弓剝離等,雖有相當發生率但在此族群病患相對來的低。本編文章著重於如何篩選,診斷及治療有合併顱動脈瘤之自體顯性多囊性腎病變病患。治療方案應取決於相關專家照會診以及針對動脈瘤病灶位置，病患年紀及其它共併症取得對病患最佳利與弊之決策。

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