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Journal of the Korean Society of Pediatric Nephrology 2003;7(2): 204-210.
막증식성 사구체신염 제 II 형(Dense-Deposit Disease) 1례
이숙진, 문재훈, 강미선, 송민섭, 정우영
1인제대학교 의과대학 부산백병원 소아과
2인제대학교 의과대학 부산백병원 소아과
3인제대학교 의과대학 부산백병원 병리과
4인제대학교 의과대학 부산백병원 소아과
5인제대학교 의과대학 부산백병원 소아과
A Case of Membranoproliferative Glomerulonephritis Type II(Dense-Deposit Disease)
Suk-Jin Lee, Jae-Hoon Moon, Mi-Seon Kang, Min-Seob Song, Woo-Yeong Chung
1Department of Pediatrics, Inje University, College of Medicine, Busan Paik Hospital
2Department of Pediatrics, Inje University, College of Medicine, Busan Paik Hospital
3Department of Pathology, Inje University, College of Medicine, Busan Paik Hospital
4Department of Pediatrics, Inje University, College of Medicine, Busan Paik Hospital
5Department of Pediatrics, Inje University, College of Medicine, Busan Paik Hospital
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ABSTRACT
Membranoproliferative glomerulonephritis type II(MPGN II), also called dense deposit disease, was first described by Berger and Galle in 1963. The diagnosis of MPGN II is based on electron-microscopic finding of an intensely electron-dense substance which replaces the lamina densa of the glomerular basement membrane. Although the etiology and pathogenesis of MPGN II are unknown, it frequently progresses to end-stage renal failure. Typically in MPGN II, hypocomplementemia due to activation of the alternative complement pathway is present. In addition, the association of MPGN II with partial lipodystrophy and complement abnormalities is well documented. The relationship between these associated features and the patient's renal functional outcome is not clear. With respect to the therapy for MPGN II, an alternate-day prednisolone regimen was shown to be effective. Various treatment modalities, including immunosuppression with corticosteroids, cytotoxic drugs and cyclosporin A, anticoagulants and antiplatelet therapies are used, either alone or in combination, with varying degrees of success. The purpose of this paper is to present a case of MPGN II from a 7 years old girl with paroxysmal supraventricular tachycardia(PSVT).
Key words: Membranoproliferative glomerulonephritis II | Dense deposit disease | Children
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