CAJ | 학술논문

目的：探讨淀粉样变性肾病( amyloid nephropathy, ANP)的临床病理学特征。方法回顾性分析31例ANP的临床病理资料,并复习相关文献。结果31例ANP占同期全部肾脏穿刺活检的1．19%(31/2603),其中女性15例,男性16例。年龄36~77岁,平均(61．28±10．95)岁。临床分期：单纯蛋白尿期4例(12．90%),肾病综合征期21例(67．74%),肾功能衰竭期6例(19．35%)。镜下见淀粉样物质主要沉积于肾小球系膜区、毛细血管基膜和小动脉壁,严重时可沉积于肾小管基膜和肾间质。高锰酸钾氧化刚果红染色示：AL型27例,AA型4例。免疫荧光检测部分病例可见免疫球蛋白及补体程度较弱的沉积,部分为阴性。 AL型ANP免疫组化标记示免疫球蛋白轻链κ和λ不同程度表达,其中以λ轻链多见。电镜下在系膜区及毛细血管壁上皮下可见淀粉样纤维。结论 ANP好发于中老年肾病患者,临床表现有时缺乏特异性,肾组织穿刺活检病理学检查是确诊的唯一方法。对疑似病例应尽早行肾脏穿刺活检,以便早期诊断及治疗。
목적：탐토정분양변성신병( amyloid nephropathy, ANP)적림상병이학특정。방법회고성분석31례ANP적림상병리자료,병복습상관문헌。결과31례ANP점동기전부신장천자활검적1．19%(31/2603),기중녀성15례,남성16례。년령36~77세,평균(61．28±10．95)세。림상분기：단순단백뇨기4례(12．90%),신병종합정기21례(67．74%),신공능쇠갈기6례(19．35%)。경하견정분양물질주요침적우신소구계막구、모세혈관기막화소동맥벽,엄중시가침적우신소관기막화신간질。고맹산갑양화강과홍염색시：AL형27례,AA형4례。면역형광검측부분병례가견면역구단백급보체정도교약적침적,부분위음성。 AL형ANP면역조화표기시면역구단백경련κ화λ불동정도표체,기중이λ경련다견。전경하재계막구급모세혈관벽상피하가견정분양섬유。결론 ANP호발우중노년신병환자,림상표현유시결핍특이성,신조직천자활검병이학검사시학진적유일방법。대의사병례응진조행신장천자활검,이편조기진단급치료。
Purpose To investigate the clinical and pathological features of amyloid nephropathy. Methods A retrospective analysis was conducted in 31 cases of amyloidosis nephropathy. The clinical data and pathologic features of kidney biopsy were analyzed. Re-sults 31 cases of amyloid degeneration accounted for 1. 19% (31/2 603) in all patients of kidney biopsy in the same period. 15 pa-tients were female, and 16 males. Patients’ age ranged from 36 to 77 years old, with mean age of (61. 28 ± 10. 95) years. Clinical staging showed that simple proteinuria were 4 cases (12. 90%), nephrotic syndrome, 21 cases (67. 74%), and renal failure, 6 cases (19. 35%). Under microscope, amyloid deposits were observed in the glomerular mesangial area, capillary basement membrane and small arteries, and those also deposited between renal interstitial and tubular basement membrane in severe cases. Potassium permanga-nate oxidation Congo red staining showed that AL type were 27 cases and AA 4 cases. Immunofluorescence study in some cases showed some degree of weak immunoglobulin and complement deposition, but some cases were negative. Immunohistochemical staining showed different expression of immunoglobulin light chain κ and λ light chains. Under electron microscope, amyloid fibrils were noted in the mesangial area and capillary walls. Conclusion Amyloidosis nephropathy occurs in middle-aged patients with kidney disease, some-times lack of specific clinical manifestations. Renal biopsy is the only approach to confirm the diagnosis. For suspicious patients, renal biopsy should be done as early as possible.