CAJ | 학술논문

目的：分析Ⅲ型胶原肾小球病的临床病理特点并探讨其相关病因、发病机制及预后。方法收集北京大学医学部病理学系从2005年至2014年收集的5例Ⅲ型胶原肾小球病的肾活检病例，进行常规光学显微镜（ HE、PASM、PAS、Masson染色）、免疫荧光（免疫球蛋白、补体的多种标记）、Ⅲ型胶原免疫组织化学染色及透射电镜检查，分析其病变及临床特点。结果Ⅲ型胶原肾小球病患者多表现为大量蛋白尿或肾病综合征；病理特点为肾小球系膜基质增生，基底膜增厚，并可出现系膜结节状硬化，各种免疫球蛋白和补体阴性，仅免疫组织化学出现Ⅲ型胶原阳性，透射电镜检查可见肾小球系膜区和基底膜大量Ⅲ型胶原沉积。其病因、发病机制不明，可能与Ⅲ型胶原代谢的基因异常有关。结论Ⅲ型胶原肾小球病较罕见，其病因和发病机制可能为Ⅲ型胶原代谢的基因异常，确诊的依据是在肾小球内发现大量Ⅲ型胶原沉积，目前缺乏有效的治疗方法，预后较差。
목적：분석Ⅲ형효원신소구병적림상병리특점병탐토기상관병인、발병궤제급예후。방법수집북경대학의학부병이학계종2005년지2014년수집적5례Ⅲ형효원신소구병적신활검병례，진행상규광학현미경（ HE、PASM、PAS、Masson염색）、면역형광（면역구단백、보체적다충표기）、Ⅲ형효원면역조직화학염색급투사전경검사，분석기병변급림상특점。결과Ⅲ형효원신소구병환자다표현위대량단백뇨혹신병종합정；병리특점위신소구계막기질증생，기저막증후，병가출현계막결절상경화，각충면역구단백화보체음성，부면역조직화학출현Ⅲ형효원양성，투사전경검사가견신소구계막구화기저막대량Ⅲ형효원침적。기병인、발병궤제불명，가능여Ⅲ형효원대사적기인이상유관。결론Ⅲ형효원신소구병교한견，기병인화발병궤제가능위Ⅲ형효원대사적기인이상，학진적의거시재신소구내발현대량Ⅲ형효원침적，목전결핍유효적치료방법，예후교차。
Objective To study the clinicopathologic features of collagen Ⅲglomerulopathy and its cause, pathogenesis and prognosis.Methods Five cases of collagen Ⅲglomerulopathy that collected from 2005 to 2014 were observed by renal biopsy.The morphologic characteristics were studied by light microscopy , immunofluorescence , immunohistochemical and electron microscopy.Results The glomerular mesangium became expansion but no hypercellularity , basement membrane appeared thickened.The glomeruli showed collagen type Ⅲdeposit by immunohistochemistry method , and collagen fibers increased by electron microscopy.The patients often show serious proteinuria , nephrotic syndrome and renal function damage.Conclusions Collagen Ⅲ glomerulopathy is an idiopathic glomerular disease , characterized by massive accumulation of collagen type Ⅲ within the glomerular mesangial areas and basement membrane.Collagen Ⅲglomerulopathy is extremely rare.The etiology and pathogenesis may relate to the abnormality of collagen Ⅲgene.There is no specific treatment for it and its prognosis is poor.