Trachtman H, Carroll F, Phadke K, Khawar M, Nicastri A, Chen C K, Tejani A
Am J Nephrol. 1987;7(1):13-7. doi: 10.1159/000167422.
It is widely believed that the most common morphological lesion in children with the idiopathic nephrotic syndrome who manifest a frequently relapsing steroid-responsive course is the minimal-change lesion. However, there are no prospective renal biopsy studies in such patients to substantiate this assertion. We performed a renal biopsy in all children with early frequently relapsing steroid-responsive nephrotic syndrome during the years 1980-1984. In 16 affected children, only 4 (25%) had minimal-change lesion, 7 had IgM nephropathy, 3 had diffuse mesangial hypercellularity, and 2 had focal segmental glomerulosclerosis. Fourteen of these patients have required immunosuppressive therapy with cyclophosphamide. Long-term follow-up revealed that 10 patients have remained protein free, 4 have persistent proteinuria despite cyclophosphamide therapy, 1 had progressed to end-stage renal disease, and 1 is lost to follow-up. On the basis of these findings, we recommend that all children with nephrotic syndrome and an early frequently relapsing steroid-responsive course undergo a prompt renal biopsy. Such patients constitute a high-risk group with a spectrum of renal histopathological lesions characterized by an unpredictable response to therapy and an unfavorable prognosis.
人们普遍认为，在表现为频繁复发的激素反应性病程的特发性肾病综合征患儿中，最常见的形态学病变是微小病变。然而，尚无针对此类患者的前瞻性肾活检研究来证实这一论断。我们在1980年至1984年期间对所有患有早期频繁复发的激素反应性肾病综合征的患儿进行了肾活检。在16名患病儿童中，只有4名（25%）患有微小病变，7名患有IgM肾病，3名患有弥漫性系膜细胞增多，2名患有局灶节段性肾小球硬化。其中14名患者需要用环磷酰胺进行免疫抑制治疗。长期随访显示，10名患者已无蛋白尿，4名患者尽管接受了环磷酰胺治疗仍持续存在蛋白尿，1名患者已进展为终末期肾病，1名患者失访。基于这些发现，我们建议所有患有肾病综合征且病程早期频繁复发且对激素有反应的患儿应及时进行肾活检。此类患者构成一个高危群体，其肾组织病理学病变范围广泛，其特点是对治疗的反应不可预测且预后不佳。
