RESUMO
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.
Assuntos
Glomerulonefrite/patologia , Glomérulos Renais/patologia , Adolescente , Membrana Basal/patologia , Biópsia , Canadá , Criança , Pré-Escolar , Complemento C3/análise , Feminino , Glomerulonefrite/complicações , Hematúria/etiologia , Humanos , Hipertensão/etiologia , Imunoglobulina A/análise , Imunoglobulina G/análise , Imunoglobulina M/análise , Masculino , Microscopia Eletrônica , Síndrome Nefrótica/etiologia , PrognósticoRESUMO
In a retrospective analysis, 51 patients with focal segmental glomerulosclerosis and idiopathic nephrotic syndrome, who were treated with steroid or cyclophosphamide therapy, were divided into three clinical groups according to the remission profile of their nephrotic syndrome. Group 1 patients (19.37%) consistently responded to medication; none has progressive renal failure (mean follow-up 10.6 years). Group 2 patients (25, 40%) failed to respond to medication; terminal renal failure has occurred in 12 of them. Group 3 patients (7, 14%) initially appeared to be responsive to medication and continued to respond for up to 18 months, but subsequently became unresponsive to any therapy; five of them have required dialysis or transplantation. This third group of patients could not be separated clinically or pathologically from group 1 patients, all of whom have an excellent prognosis. One should, therefore, be cautious about predicting the outcome of steroid-responsive nephrotic patients, especially those with FSGS, until at least 18 months after the onset of illness.