J Korean Soc Pediatr Nephrol.  2003 Oct;7(2):204-210.

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

• Lee SJ
• Moon JH
• Kang MS
• Song MS
• Chung WY

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).

Keyword

Membranoproliferative glomerulonephritis II; Dense deposit disease; Children

MeSH Terms

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
Adrenal Cortex Hormones
Anticoagulants
Complement System Proteins
Cyclosporine
Prednisolone