Clinical Implication of Tolvaptan in Patients with Autosomal Dominant Polycystic Kidney Disease

Semin Cho, J. Paek, W. Park, K. Jin, D. K. Kim, Seungyeup Han, Yaerim Kim
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Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disease characterized by the growth of numerous fluid-filled cysts in the kidneys. It leads to progressive kidney enlargement and end-stage kidney disease (ESKD) [1,2]. ADPKD is a ciliopathy that involves abnormal cilia structure and function [3]. There are polycystin-1 and polycystin-2 in the kidney tubular cell protruding primary cilium. It has a role in detecting fluid flow and regulating calcium influx that activates an intracellular calcium signaling pathway [4]. In addition, increased levels of arginine vasopressin levels induced increased intracellular adenosine cyclic monophosphate (cAMP) levels in the distal tubule and collecting duct. Tolvaptan is a vasopressin receptor antagonist; it selectively blocks the binding of V2 receptors in tubular cells and reduces fluid secretion, cell proliferation, and cyst development by reducing cAMP [5]. Tolvaptan in ADPKD papISSN 2092-8335 · eISSN 2733-5380 Keimyung Med J [Epub ahead of print] https://doi.org/10.46308/kmj.2022.00059 Original Article
托伐普坦治疗常染色体显性多囊肾病的临床意义
常染色体显性多囊肾病(ADPKD)是最常见的遗传性肾脏疾病,其特征是肾脏中大量充满液体的囊肿的生长。它导致进行性肾脏增大和终末期肾脏疾病(ESKD)[1,2]。ADPKD是一种纤毛结构和功能异常的纤毛病[3]。肾小管细胞中有多囊蛋白1和多囊蛋白2,突出初级纤毛。它具有检测流体流动和调节钙内流的作用,激活细胞内钙信号通路[4]。此外,精氨酸加压素水平升高诱导远端小管和集管细胞内环单磷酸腺苷(cAMP)水平升高。托伐普坦是一种抗利尿激素受体拮抗剂;它选择性阻断小管细胞中V2受体的结合,通过降低cAMP减少液体分泌、细胞增殖和囊肿发育[5]。Tolvaptan in ADPKD papISSN 2092-8335·eISSN 2733-5380 Keimyung Med J [Epub预印]https://doi.org/10.46308/kmj.2022.00059原创文章
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