Rambausek M, Waldherr R, Rauterberg W, Andrassy K, Ritz E
Department of Internal Medicine, Pathology and Immunology, University of Heidelberg, FRG.
Nephron. 1987;47(3):190-3. doi: 10.1159/000184489.
A 17-year-old male presented with nephrotic syndrome associated with microscopic hematuria. Renal biopsy showed only minor glomerular abnormalities (light microscopy). Immunohistology demonstrated strong mesangial deposition of IgA. Electronmicroscopy disclosed widespread effacement of foot processes in combination with isolated osmiophilic mesangial deposits. The patient responded to standard corticosteroid therapy with complete disappearance of proteinuria. Microscopic hematuria, however, persisted. Five months after steroid therapy was stopped, the nephrotic syndrome relapsed. It was again steroid-responsive with persisting microhematuria. From clinical and morphological data we conclude that the patient has concomitant idiopathic nephrotic syndrome (minimal change glomerulonephritis) and mesangial IgA glomerulonephritis. The simultaneous presence of these two diseases may give some hint as to their pathogenesis. In both, abnormalities in T cell regulation have been found. If these were indeed involved in the pathogenesis of the two glomerular diseases, a higher than expected probability for the two entities to coexist in the same patient is to be expected.
一名17岁男性患者出现肾病综合征并伴有镜下血尿。肾活检仅显示轻微的肾小球异常（光镜检查）。免疫组织学显示IgA在系膜区有强烈沉积。电镜检查发现足突广泛消失，并伴有孤立的嗜锇性系膜沉积物。患者对标准皮质类固醇治疗有反应，蛋白尿完全消失。然而，镜下血尿持续存在。停用类固醇治疗5个月后，肾病综合征复发。再次使用类固醇治疗有效，但镜下血尿仍持续。根据临床和形态学数据，我们得出结论，该患者同时患有特发性肾病综合征（微小病变性肾小球肾炎）和系膜IgA肾小球肾炎。这两种疾病的同时存在可能为它们的发病机制提供一些线索。在这两种疾病中，均发现了T细胞调节异常。如果这些异常确实参与了两种肾小球疾病的发病机制，那么这两种疾病在同一患者中共存的概率可能会高于预期。
