CAJ | 학술논문

目的：探讨肾脏淀粉样变性病(amyloidosis,AL)的临床病理特点、诊断及鉴别诊断。方法回顾性分析9例肾脏淀粉样变性病患者的临床表现,并对其肾活检组织进行光镜、特殊染色、免疫荧光、免疫组化及超微结构观察。同时对患者进行随访获取预后信息。结果9例肾脏淀粉样变性病患者的临床上主要表现为肾病综合征伴进行性肾功能不全并逐渐恶化。肾活检组织光镜观察,早期淀粉样变光镜表现轻微,可有系膜轻度增生或基底膜空泡变性及轻度增厚,PASM染色可见节段性睫毛状结构；后期,肾小球系膜区明显增宽、基底膜增厚,毛细血管腔闭塞,呈无细胞结节硬化状态。免疫荧光表现不一,部分病例全部阴性,部分表现免疫球蛋白及补体沿系膜区或毛细血管壁不同程度的沉积。刚果红染色呈砖红色,甲基紫染色呈紫红色。免疫组化显示,5例来源于轻链λ,1例来源于轻链κ,全阴性者有3例。电镜观察下,肾小球毛细血管基底膜部分节段无明显病变,部分节段不规则增厚,肾小球基底膜外侧和(或)内皮下可见淀粉样纤维沉积；系膜轻、中度无细胞性增生,其内可见不规则排列淀粉样纤维沉积,直径8~10 nm,无分支,僵硬,排列紊乱。足突大部分融合,肾间质纤维化,其内亦可见淀粉样纤维沉积。结论肾脏淀粉样变性多见于中老年,根据光镜的典型病理学改变,并结合刚果红和甲基紫染色,可明确诊断。电镜检查是早期诊断肾淀粉样变的重要手段。
목적：탐토신장정분양변성병(amyloidosis,AL)적림상병리특점、진단급감별진단。방법회고성분석9례신장정분양변성병환자적림상표현,병대기신활검조직진행광경、특수염색、면역형광、면역조화급초미결구관찰。동시대환자진행수방획취예후신식。결과9례신장정분양변성병환자적림상상주요표현위신병종합정반진행성신공능불전병축점악화。신활검조직광경관찰,조기정분양변광경표현경미,가유계막경도증생혹기저막공포변성급경도증후,PASM염색가견절단성첩모상결구；후기,신소구계막구명현증관、기저막증후,모세혈관강폐새,정무세포결절경화상태。면역형광표현불일,부분병례전부음성,부분표현면역구단백급보체연계막구혹모세혈관벽불동정도적침적。강과홍염색정전홍색,갑기자염색정자홍색。면역조화현시,5례래원우경련λ,1례래원우경련κ,전음성자유3례。전경관찰하,신소구모세혈관기저막부분절단무명현병변,부분절단불규칙증후,신소구기저막외측화(혹)내피하가견정분양섬유침적；계막경、중도무세포성증생,기내가견불규칙배렬정분양섬유침적,직경8~10 nm,무분지,강경,배렬문란。족돌대부분융합,신간질섬유화,기내역가견정분양섬유침적。결론신장정분양변성다견우중노년,근거광경적전형병이학개변,병결합강과홍화갑기자염색,가명학진단。전경검사시조기진단신정분양변적중요수단。
Objective To study the clinicopathological features,diagnosis and differential diag-nosis of renal amyloidosis.Methods Nine cases of renal amyloidosis were studied by means of clinical and follow-up data,light microscopy (LM),immunofluorescense (IF),immunohistochemistry (IHC) and electron microscopy (EM).Results The average age of patients with renal amyloidosis was (52.5 ±7.8)years (mean 43-62).Nephrotic syndrome was the most prominent symptoms accompanied with progressive renal insufficiency and conditions grew worsening.In the early stage,pathological ex-aminations of renal biopsies using LM and IF showed mild mesangial proliferation and mild thickening of glomerular basement membrane (GBM).Segmental eyelash structure could be seen by PASM stai-ning.In the advanced stage,glomerular mesangial area widened significantly,and basement membrane thickened.Capillary lumen was occluded,and no cell nodular sclerosis occurred.Immunoglobulins and complements were negative or only scanty in certain cases.Congo red staining and methyl violet stai-ning were positive.IHC revealed 5 cases from the light chainλ,1 from the light chainκ,and 3 cases were whole negative.The amyloid fibrils were randomly distributed in the mesangium,along GBM and at the arteriolar wall under EM.Conclusions Renal amyloidosis predominantly occurs in the elderly. Renal amyloidosis could be clearly diagnosed by typical pathological changes of LM,in combination with Congo red and methyl violet staining.EM observation is an indispensable technique for the diag-nosis of early renal amyloidosis.