특발성 제2형 막증식성 사구체신염 2예

Abstract

The term type II membranoproliferarive gkr merulonephritis (MPGN) refers to the histopathologic entity characterized by dense intramembranous deposits. It have a variable clincal courses, frequently occurs in older children and young adult. In comparison with The western, the idiopathic membrano-proliferative glomerulonephritis (MPGN) has a lower frequency than secondary MPGN. Especially, of the idiopathic MPGK, the frequency of type 2 MPGN, so called dense deposit disease, is very rare in Korea. We are reporting two cases of type II MPGN, which was proven by renal biopsy. The clinical manifestations were recurrent gross hematuria in one patient and persistent nephrotic -ranged proteinuria in the other patient. The biopsy findings are characterized by diffuse wall thickening of capillary walls and focal proliferation of mesangial cell in light microscopy, and by capillary wall and granular basement membrane staining of C3 in immunofluorescence mr croscopy, and an irregular fusiform swelling of the lamina densa which resulting in a further thickening of basement of basement membrane in electron mi-croscopy. Our two patients were treated conserva-tively without using steroid or immunosuppressive agents. One patient who had followed-up for 7 years after diagnosis remain stable in renal function, and the other patient who had folbwedmp for 4 years after diagnosis showed persistent nephrotic-range proteinuria.