A Case of Membranoproliferative Glomerulonephritis Type II (Dense-Deposit Disease)

Lee, Suk-Jin; Moon, Jae-Hoon; Kang, Mi-Seon; Song, Min-Seob; Chung, Woo-Yeong

J Korean Soc Pediatr Nephrol > Volume 7(2); 2003 > Article

J Korean Soc Pediatr Nephrol 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

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