Sanders P W
Department of Medicine, University of Alabama at Birmingham, 35294-0007.
J Lab Clin Med. 1994 Oct;124(4):484-8.
The focus of therapy of cast nephropathy, or "myeloma kidney," has been chemotherapy to decrease production of the abnormal monoclonal immunoglobulin and immunoglobulin light chain, known as Bence Jones protein. Little attention has been given to understanding and disrupting the pathophysiologic mechanisms involved in production of intraluminal casts that obstruct and ultimately destroy renal function. Myeloma casts develop in the distal nephron when cast-forming light chains bind to a specific portion of Tamm-Horsfall glycoprotein, secreted by cells of the thick ascending limb of the loop of Henle, to form an insoluble protein complex. A variety of factors including tubule fluid flow rate and concentrations of calcium and sodium chloride in the distal nephron modify the interactions between these proteins and thus influence subsequent clinical renal failure. This review summarizes the latest developments in the pathogenesis and management of cast nephropathy.
管型肾病，即“骨髓瘤肾病”的治疗重点一直是化疗，以减少异常单克隆免疫球蛋白和免疫球蛋白轻链（即本-周蛋白）的产生。对于理解和破坏参与形成阻塞并最终破坏肾功能的管腔内管型的病理生理机制，关注甚少。当形成管型的轻链与亨利氏袢升支粗段细胞分泌的Tamm-Horsfall糖蛋白的特定部分结合，形成不溶性蛋白质复合物时，骨髓瘤管型在远端肾单位中形成。包括肾小管液流速以及远端肾单位中钙和氯化钠浓度在内的多种因素会改变这些蛋白质之间的相互作用，从而影响随后的临床肾衰竭。本综述总结了管型肾病发病机制和管理方面的最新进展。
