Habib R, Niaudet P
Pediatric Nephrology Unit, Hôpital Necker-Enfants Malades, Paris, France.
Clin Nephrol. 1994 Sep;42(3):141-6.
Fourty-two children with idiopathic nephrosis that had not been well controlled by other forms of therapy were treated with ciclosporine. Thirty-three of them were steroid dependent, 2 were partial steroid responders and 7 were steroid resistant. On pretreatment renal biopsy, performed in all patients less than 6 months before starting ciclosporine, minimal change disease (MCD) was diagnosed in 37 children and focal glomerular sclerosis (FGS) in 5. In order to evaluate the morphological changes of the renal parenchyma possibly induced by the drug, posttreatment biopsies were performed in these 42 patients: one in all patients after 4 to 28 months of ciclosporine, 2 in 23 patients after 18 to 42 months of treatment, 3 in 8 patients after 30 to 63 months of treatment and 4 in 2 patients who had been treated respectively for 62 and 63 months. The morphological changes on pre- and posttreatment biopsies were scored according to the severity of tubulointerstitial lesions. Grade I was considered when there were no significant changes of the renal parenchyma or when occasional scattered tubules with thickened basement membranes were present. Grade II was diagnosed when the biopsy showed several small foci of atrophic tubules with thickened basement membranes within stripes of interstitial fibrosis and grade III when confluent or extensive areas of interstitial fibrosis with atrophic and/or collapsed tubules were observed. On pretreatment renal biopsy, only one patient showed tubulointerstitial lesions (grade II). On the latest biopsy obtained, 18 patients showed grade I tubulointerstitial lesions, 15 grade II and 9 grade III. Unspecific arteriolopathy was observed in 10 patients.(ABSTRACT TRUNCATED AT 250 WORDS)
42名患有特发性肾病且其他治疗方式未能有效控制病情的儿童接受了环孢素治疗。其中33名对类固醇依赖,2名对类固醇部分反应,7名对类固醇抵抗。在开始使用环孢素治疗前不到6个月,所有患者均进行了肾活检,37名儿童被诊断为微小病变肾病(MCD),5名被诊断为局灶性肾小球硬化(FGS)。为了评估该药物可能引起的肾实质形态学变化,对这42名患者进行了治疗后活检:4至28个月环孢素治疗后所有患者均进行了1次活检,18至42个月治疗后23名患者进行了2次活检,30至63个月治疗后8名患者进行了3次活检,分别接受了62个月和63个月治疗的2名患者进行了4次活检。根据肾小管间质病变的严重程度对治疗前后活检的形态学变化进行评分。当肾实质无明显变化或偶尔出现散在的基底膜增厚的肾小管时,评为I级。当活检显示在间质纤维化条带内有几个萎缩性肾小管的小病灶且基底膜增厚时,诊断为II级,当观察到融合或广泛的间质纤维化区域伴有萎缩和/或塌陷的肾小管时,评为III级。治疗前肾活检时,只有1名患者出现肾小管间质病变(II级)。在最新获得的活检中,18名患者显示I级肾小管间质病变,15名显示II级,9名显示III级。10名患者观察到非特异性小动脉病变。(摘要截断于250字)