结晶型轻链近端肾小管病  

Crystalline light chain proximal tubulopathy

作　　者：曾梦平 朱雪婧[1] 刘虹[1] ZENG Mengping;ZHU Xuejing;LIU Hong(Department of Nephrology,The Second Xiangya Hospital of Central South University,Changsha 410011,China)

机构地区：[1]中南大学湘雅二医院肾内科中南大学肾脏病研究所,南京210016

出　　处：《肾脏病与透析肾移植杂志》2023年第6期590-594,共5页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：中南大学创新创业教育课程建设项目(2020CXKZ02);2022年中南大学教育教学改革研究项目(2022jy160)。

摘　　要：43岁男性,既往发现血清肌酐升高1年余。肾脏损害表现为蛋白尿和肾功能不全,伴Fanconi综合征,血和尿免疫固定电泳κ阳性,血κ/λ比值明显升高。肾活检病理提示急性肾小管损伤,免疫电镜可见单克隆κ阳性轻链结晶沉积于肾小管胞质。最终诊断为轻链近端肾小管病(结晶型)。A 43-year-old man presented with recurrent creatinine elevation for one year.The clinical manifestations were proteinuria and renal damage,accompanied with Fanconi syndrome.Immunofixation electrophoresis indicated kappa light chain band in blood and urine,and the ratio ofκ/λwas significantly increased.Renal biopsy pathology revealed acute tubular injury.Immunoelectron microscopy show monoclonal kappa light chain crystals deposited in renal tubular cytoplasm.The final diagnosis was light chain proximal tubulopathy(crystalline type).

关 键 词：轻链近端肾小管病 FANCONI综合征 急性肾小管损伤 具有肾脏意义的单克隆免疫球蛋白病 肾小管酸中毒 

分 类 号：R692[医药卫生—泌尿科学]