MPGN II/DDD Information for Physicians

Dense Deposit Disease / Membranoproliferative Glomerulonephritis Type II: Highlights

DDD/MPGNII is a rare disease. It is primarily diagnosed in children between 5-15 years of age. The disease is equally represented among sexes. Within 10 years of diagnosis, end-stage renal disease develops in about 50% of these children.

The diagnosis of DDD/MPGNII requires renal biopsy. The biopsy will show dense deposits in the glomerular basement membrane on electron microscopy. Eye problems can develeop and so persons with DDD/MPGNII should see an eye doctor.

The cause of DDD/MPGNII appears to be related to uncontrolled systemic activation of the alternative pathway of the complement cascade.

Most current treatments for DDD/MPGNII are ineffective. However, some persons with DDD/MPGNII do have mutations in a gene called Factor H (CFH). For these patients, plasma exchange can control complement activation and prevent end stage renal disease. Genetic screening of factor H should be offered to all patients with DDD/MPGNII.

For an in depth review of DDD/MPGNII please see

Appel et al. Membranoproliferative Glomerulonephritis Type II (Dense Deposit Disease): An Update. J Am Nephrol Soc 16: 1392-1403, 2005.

Zipfel PF, Smith RJH, Heinen S. The role of complement in membranoproliferative glomerulonephritis. In Zipfel PF, ed. Complement and Kidney Disease. Birkhauser Verlag Basel, Switzerland, 2006.

Smith RJ, Sethi S, Zipfel PF. Dense Deposit/ DiseaseMembranoproliferative Glomerulonephritis Type II In: /GeneReviews /at GeneTests: Medical Genetics Information Resource [database online]. Copyright, University of Washington, Seattle. At www.genetests.org <http://www.geneclinics.org/> 2007.



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	Dense Deposit Disease / Membranoproliferative Glomerulonephritis Type II: Highlights DDD/MPGNII is a rare disease. It is primarily diagnosed in children between 5-15 years of age. The disease is equally represented among sexes. Within 10 years of diagnosis, end-stage renal disease develops in about 50% of these children. The diagnosis of DDD/MPGNII requires renal biopsy. The biopsy will show dense deposits in the glomerular basement membrane on electron microscopy. Eye problems can develeop and so persons with DDD/MPGNII should see an eye doctor. The cause of DDD/MPGNII appears to be related to uncontrolled systemic activation of the alternative pathway of the complement cascade. Most current treatments for DDD/MPGNII are ineffective. However, some persons with DDD/MPGNII do have mutations in a gene called Factor H (CFH). For these patients, plasma exchange can control complement activation and prevent end stage renal disease. Genetic screening of factor H should be offered to all patients with DDD/MPGNII. For an in depth review of DDD/MPGNII please see Appel et al. Membranoproliferative Glomerulonephritis Type II (Dense Deposit Disease): An Update. J Am Nephrol Soc 16: 1392-1403, 2005. Zipfel PF, Smith RJH, Heinen S. The role of complement in membranoproliferative glomerulonephritis. In Zipfel PF, ed. Complement and Kidney Disease. Birkhauser Verlag Basel, Switzerland, 2006. Smith RJ, Sethi S, Zipfel PF. Dense Deposit/ DiseaseMembranoproliferative Glomerulonephritis Type II In: /GeneReviews /at GeneTests: Medical Genetics Information Resource [database online]. Copyright, University of Washington, Seattle. At www.genetests.org <http://www.geneclinics.org/> 2007.		Factor H Genetic Testing Genetic testing for factor H is offered through the MORL. Information sheet Sending a blood sample for screening Clinical data sheet
Contact us			Useful References On-line renal pathology CME courses including MPGNII/DDD at: www.gamewood.net http://path.upmc.edu The National Organization for Rare Disorders entry on MPGNII/DDD at: www.rarediseases.org An E-Medicine information page on MPGN for Physicians at: www.emedicine.com