Lago N R, Bulos M J, Monserrat A J
Departamento de Patología, Facultad de Medicina, Universidad de Buenos Aires, Argentina.
Medicina (B Aires). 1997;57(2):213-5.
Fibrillar collagen in the glomeruli is considered specific of the nail-patella syndrome. A new nephropathy with diffuse intraglomerular deposition of type III collagen without nail and skeletal abnormalities has been described. We report the case of a 26-year-old woman who presented persistent proteinuria, hematuria, deafness without nail and skeletal abnormalities. The renal biopsy showed focal and segmental glomerulosclerosis by light microscopy. The electron microscopy revealed the presence of massive fibrillar collagen within the mesangial matriz and the basement membrane. This is the first patient reported in our country. We emphasize the usefulness of electron microscopy in the study of glomerular diseases.
肾小球中的纤维状胶原蛋白被认为是指甲-髌骨综合征的特异性表现。已有文献描述了一种新的肾病，其肾小球内弥漫性III型胶原蛋白沉积，且无指甲和骨骼异常。我们报告了一例26岁女性病例，该患者存在持续性蛋白尿、血尿、耳聋，无指甲和骨骼异常。肾活检光镜检查显示局灶节段性肾小球硬化。电镜检查发现系膜基质和基底膜内存在大量纤维状胶原蛋白。这是我国报道的首例此类患者。我们强调电镜检查在肾小球疾病研究中的实用性。
