CAJ | 학술논문

多囊肾病是常见的遗传性肾脏疾病，按遗传方式可分为常染色体显性多囊肾病和常染色体隐性多囊肾病。最常见的多囊肾病是常染色体显性多囊肾病，本文分析的多囊肾病特指常染色体显性多囊肾病。对于多囊肾病，囊肿的生长和肾功能丧失均与进展性纤维化有关。肾脏纤维化是各种肾病进展至终末期肾病的重要机制，增加了终末期肾病的发生率，但目前其潜在的发生机制尚未明确。在多囊肾病中，肾脏纤维化的机制包括转化生长因子-β表达上调、间质胶原蛋白表达增加、上皮间质转化及纤溶酶原激活物抑制剂表达增多等。鉴于肾脏纤维化在多囊肾病中的重要作用，阻止或减缓肾脏纤维化能减缓多囊肾病的进展；而有效的抗纤维化治疗依赖于对多囊肾病中肾脏纤维化确切发病机制的了解。本文旨在讨论多囊肾病的进展与肾脏纤维化的关系。
다낭신병시상견적유전성신장질병，안유전방식가분위상염색체현성다낭신병화상염색체은성다낭신병。최상견적다낭신병시상염색체현성다낭신병，본문분석적다낭신병특지상염색체현성다낭신병。대우다낭신병，낭종적생장화신공능상실균여진전성섬유화유관。신장섬유화시각충신병진전지종말기신병적중요궤제，증가료종말기신병적발생솔，단목전기잠재적발생궤제상미명학。재다낭신병중，신장섬유화적궤제포괄전화생장인자-β표체상조、간질효원단백표체증가、상피간질전화급섬용매원격활물억제제표체증다등。감우신장섬유화재다낭신병중적중요작용，조지혹감완신장섬유화능감완다낭신병적진전；이유효적항섬유화치료의뢰우대다낭신병중신장섬유화학절발병궤제적료해。본문지재토론다낭신병적진전여신장섬유화적관계。
Polycystic kidney disease( PKD),a common hereditary kidney disease,can be divided,according to mode of inheritance,into autosomal dominant and autosmal recessive,and autosomal dominant PKD is the commonest,which will be analyzed specifically in this paper. For PKD,renal cyst growth and renal functional incapacitation is associated with pro-gressive fibrosis. Renal fibrosis,an important mechanism of progression-to-end-stage kidney disease( KD),leads to inci-dence of end-stage KD,the mechanism of which remains unknown. In PKD,the mechanisms of renal fibrosis include increas-ing expressions of transforming growth factor-β,interstitial collagen protein,epithelial-mesenchymal transition and plasmino-gen activator inhibitor and so on. Given the important role of renal fibrosis in PKD,preventing or slowing renal fibrosis can retard PKD progression. Effective anti-fibrosis therapy depends on the understanding of renal fibrosis mechanism. This paper is aimed to discuss the relationship PKD progression to renal fibrosis.