A Case of Membranoproliferative Glomerulonephritis Type II (Dense-Deposit Disease).
- Author:
Suk Jin LEE
1
;
Jae Hoon MOON
;
Mi Seon KANG
;
Min Seob SONG
;
Woo Yeong CHUNG
Author Information
1. Department of Pediatrics, Inje University, College of Medicine, Busan Paik Hospital, Busan, Korea.
- Publication Type:Case Report
- Keywords:
Membranoproliferative glomerulonephritis II;
Dense deposit disease;
Children
- MeSH:
Adrenal Cortex Hormones;
Anticoagulants;
Child;
Complement Pathway, Alternative;
Complement System Proteins;
Cyclosporine;
Diagnosis;
Female;
Glomerular Basement Membrane;
Glomerulonephritis, Membranoproliferative*;
Humans;
Immunosuppression;
Kidney Failure, Chronic;
Lipodystrophy;
Prednisolone
- From:Journal of the Korean Society of Pediatric Nephrology
2003;7(2):204-210
- CountryRepublic of Korea
- Language:Korean
-
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).
