Latham P, Poucell S, Koresaar A, Arbus G, Baumal R
J Pediatr. 1982 Nov;101(5):682-5. doi: 10.1016/s0022-3476(82)80290-4.
The 1,205 renal biopsies performed at The Hospital for Sick Children, Toronto, were reviewed to identify membranous glomerulopathy. Fourteen patients had a clinicopathologic diagnosis of idiopathic membranous glomerulopathy. Typical thickening of glomerular capillary basement membranes, a spike-and-dome pattern, and subepithelial electron-dense deposits were noted. Strong deposits of IgG and weaker deposits of C3, IgM, and IgA were present in glomeruli. Stages of membranous glomerulopathy on electron microscopy were I in one biopsy, II in nine biopsies, and III in four biopsies. Two additional biopsies from one child initially showed minimal lesion-type disease; later, a third showed membranous glomerulopathy. At presentation 11 patients had nephrotic syndrome, seven had hypertension, and eight had hematuria. Now four are in remission, seven have active disease with normal renal function, and three have renal failure. Patients with hypertension tended to do worse than those without. Age at onset, presence of nephrotic syndrome or hematuria, and administration of steroids or immunosuppressive drugs did not adversely affect outcome. Furthermore, clinical outcome did not correlate with stage of disease. Hence pathologic and most clinical features do not predict long-term prognosis in children with membranous glomerulopathy.
回顾了在多伦多病童医院进行的1205例肾活检,以确定膜性肾小球病。14例患者经临床病理诊断为特发性膜性肾小球病。可见肾小球毛细血管基底膜典型增厚、钉突和穹窿样改变以及上皮下电子致密沉积物。肾小球内存在强阳性的IgG沉积物以及弱阳性的C3、IgM和IgA沉积物。电镜下膜性肾小球病的分期为:1例活检为I期,9例为II期,4例为III期。一名儿童的另外两次活检最初显示为微小病变型疾病;后来,第三次活检显示为膜性肾小球病。就诊时,11例患者患有肾病综合征,7例患有高血压,8例患有血尿。目前,4例缓解,7例肾功能正常但疾病活动,3例肾衰竭。有高血压的患者往往比没有高血压的患者情况更差。发病年龄、肾病综合征或血尿的存在以及类固醇或免疫抑制药物的使用对预后没有不利影响。此外,临床结果与疾病分期无关。因此,病理和大多数临床特征不能预测儿童膜性肾小球病的长期预后。