Semin Cho, J. Paek, W. Park, K. Jin, D. K. Kim, Seungyeup Han, Yaerim Kim
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引用次数: 0
摘要
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
Clinical Implication of Tolvaptan in Patients with Autosomal Dominant Polycystic Kidney Disease
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