COLLAGEN TYPE-III GLOMERULOPATHY - A NEW IDIOPATHIC GLOMERULAR-DISEASE
COLLAGEN TYPE-III GLOMERULOPATHY - A NEW IDIOPATHIC GLOMERULAR-DISEASE
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DOI：
10.1159/000168350
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发表时间：
1991-09-01
影响因子：
4.2
通讯作者：
MIHATSCH, MJ
中科院分区：
文献类型：
--
作者：
IMBASCIATI, E;GHERARDI, G;MIHATSCH, MJ
报道了一名49岁的意大利妇女的一种新型特发性肾小球疾病，她表现出不典型的肾脏症状，即，高血压和轻微蛋白尿。临床研究排除了家族性肾脏疾病，更具体地说，排除了甲-髌骨综合征。光镜下诊断性肾活检显示与膜增生性肾小球肾炎相似的图像。增大的肾小球呈分叶状，周围基底膜增厚，光镜下可见不明确的物质沉积，细胞增殖缺乏。通过电子显微镜，材料是不均匀的，部分颗粒状部分纤维状，含有典型的胶原纤维。后者被鉴定为III型胶原，在较小程度上为I型胶原。文献综述发现日本报告了12例相似或相同病例，另有1例白色美国女性报告。缺乏系统性疾病的证据。病因和发病机制是难以捉摸的。必须预期肾功能的进行性恶化。III型胶原肾小球病被认为是这种新型特发性肾小球疾病的术语。
A new type of idiopathic glomerular disease is reported in a 49-year-old Italian woman who presented with uncharacteristic renal symptoms, i.e., hypertension and slight proteinuria. Clinical investigation excluded a familial renal disease and more specifically nail-patella syndrome. Diagnostic renal biopsy by light microscopy showed a picture similar to membranoproliferative glomerulonephritis. The enlarged glomeruli were lobulated, the peripheral basement membranes were thickened by the deposition of light-microscopically undefined material, cell proliferation was lacking. By electron microscopy, the material was nonhomogenous, partly granular partly fibrillar, containing typical collagen fibers. The latter were identified as collagen type III, to a lesser extent collagen type I. Review of the literature resulted in 12 similar or identical cases reported from Japan and one additional case reported in a white American female. Evidence of systemic disease is lacking. Etiology and pathogenesis are elusive. A progressive deterioration of renal function must be expected. Collagen type III glomerulopathy is suggested as term of this new type of idiopathic glomerular disease.