Abstract：
Light and electron microscopic as well as immunofluorescent findings in kidney biopsies of 9 patients suffering from nephronophthisis are reported. The lightmicroscopic picture is unspecific except the cysts at the cortico-medullary junction which are present in 70% of cases. The immunofluorescent findings are either negative or unspecifically positive. On electron microscopy, the tubular basement membranes exhibit characteristic but not specific changes, i.e. homogeneous thickening, splitting, reticulation, thinning, complete loss, granular disintegration and collapse. These findings suggest that the \"Anlageschwäche\" of the nephron especially of the tubular basement membrane may be the primary lesion in nephronophthisis.
摘要：
据报道，9名患有肾单位的患者的肾脏活检中的光镜和电子显微镜以及免疫荧光发现。除了70％的病例中存在的皮质-髓质交界处的囊肿外，光显微照片没有特异性。免疫荧光发现是阴性或非特异性阳性。在电子显微镜上，管状基底膜表现出特征性的变化，但不是特定的变化，即均匀增厚，分裂,网状,变薄，完全损失,颗粒崩解和塌陷。这些发现表明，肾单位，尤其是肾小管基底膜的“Anlageschwäche”可能是肾单位的主要病变。
