Zurawski Jakub, Salwa-Zurawska Wiesława, Woźniak Aldona, Bortkiewicz Elzbieta, Maciejewski Janusz, Idasiak-Piechocka Ilona, Urbański Bartosz
Department of Clinical Pathomorphology, Karol Marcinkowski Medical University in Poznań, Poznań.
Pol J Pathol. 2009;60(1):35-42.
The report presents the results of investigations carried out in 63 patients (49 children and 14 adults) with thin glomerular basement membrane disease. Of 49 children, 13 came from nine families with family members suffering from kidney diseases (mostly manifested by haematuria). In the group of children, the most frequent initial clinical symptom (noted in 29 cases) was isolated haematuria, more rarely (in eight cases) haematuria and proteinuria, and (in seven cases) nephrotic syndrome. Isolated proteinuria was observed in another three children. In the adults, only in five patients was isolated haematuria the initial symptom of the disease; more frequently (in seven cases), they presented with isolated proteinuria. In two patients, proteinuria and haematuria were noted. In the two groups of patients, in addition to markers of thin glomerular basement membrane disease, the authors also observed markers indicative of other glomerulopathies: in seven children and four adults focal segmental glomerulosclerosis (FSGS) without any other glomerular pathologies (with the exception of thin glomerular basement membrane disease). Also in cases in which in addition to thin glomerular basement membrane disease other glomerular pathologies were present (mesangial hypercellularity, mesangial glomerulonephritis), matrix expansion was detected; this phenomenon might be considered a harbinger of glomerular sclerosis. In general, although not in each and every case, these pathologies were associated with duration of the disease.
该报告展示了对63例薄肾小球基底膜病患者（49名儿童和14名成人）进行调查的结果。在49名儿童中，有13名来自九个有家庭成员患有肾脏疾病（主要表现为血尿）的家庭。在儿童组中，最常见的初始临床症状（29例）是孤立性血尿，较少见的（8例）是血尿和蛋白尿，以及（7例）肾病综合征。另外三名儿童观察到孤立性蛋白尿。在成人中，只有五名患者的初始症状是孤立性血尿；更常见的（7例）是孤立性蛋白尿。两名患者出现蛋白尿和血尿。在两组患者中，除了薄肾小球基底膜病的标志物外，作者还观察到了提示其他肾小球病的标志物：7名儿童和4名成人患有局灶节段性肾小球硬化（FSGS），无任何其他肾小球病变（薄肾小球基底膜病除外）。此外，在除薄肾小球基底膜病外还存在其他肾小球病变（系膜细胞增多、系膜增生性肾小球肾炎）的病例中，检测到基质扩张；这种现象可能被认为是肾小球硬化的先兆。一般来说，虽然并非在每一个病例中都是如此，但这些病变与疾病持续时间有关。
