Dense deposit disease is a rare but devastating disease primarily affecting children. This review focuses on new information regarding the pathophysiology of dense deposit disease, its appearance histopathologically, its relationship to other diseases including macular degeneration and acquired partial lipodystrophy and potential new therapies. The microscopic features of dense deposit disease have been separated into five patterns with only about 25% of patients showing membranoproliferative features. The subtle interplay between genetic changes in complement regulatory proteins and dysregulation of the alternative pathway of complement is now more evident. Haplotype mapping has shown at-risk phenotypes of complement factor H associated with the development of dense deposit disease. Treatment protocols are empiric and not very effective. New information on complement inhibitors and plasma exchange, however, has brought hope for new therapies in the near future. Understanding of the pathology and the pathophysiology of dense deposit disease has advanced rapidly in the last decade. New efforts in genetic mapping along with the development of novel inhibitors of the complement system will lead to improved care for patients afflic...Continue Reading
Transplantation in mesangiocapillary glomerulonephritis with intramembranous dense "deposits": recurrence of disease
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Regression of membranoproliferative glomerulonephritis type II (dense deposit disease): observations in six children
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Hereditary porcine membranoproliferative glomerulonephritis type II is caused by factor H deficiency
Primary culture of rabbit proximal tubules as a cellular model to study nephrotoxicity of xenobiotics
Porcine membranoproliferative glomerulonephritis type II: an autosomal recessive deficiency of factor H
Investigation of mechanism-based inhibitors of complement targeting the activated thioester of human C3
Outcome of idiopathic membranoproliferative glomerulonephritis in children. Arbeitsgemeinschaft Pädiatrische Nephrologie
Human factor H deficiency. Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism.
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Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II ("dense deposit disease")
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Effect of eculizumab on hemolysis and transfusion requirements in patients with paroxysmal nocturnal hemoglobinuria
A rare variant in CFH directly links age-related macular degeneration with rare glomerular nephropathies.
Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation
Autoimmune forms of thrombotic microangiopathy and membranoproliferative glomerulonephritis: Indications for a disease spectrum and common pathogenic principles
Expression of human complement factor H prevents age-related macular degeneration-like retina damage and kidney abnormalities in aged Cfh knockout mice.
Translational mini-review series on complement factor H: therapies of renal diseases associated with complement factor H abnormalities: atypical haemolytic uraemic syndrome and membranoproliferative glomerulonephritis.
Recurrent dense deposit disease after renal transplantation: an emerging role for complementary therapies
Relationship of complement activation route with clinical manifestations in Japanese patients with systemic lupus erythematosus: a retrospective observational study
Positive C1q staining associated with poor renal outcome in membranoproliferative glomerulonephritis
Alternative Complement Pathway
The Alternative Complement Pathway is part of the innate immune system, and activation generates membrane attack complexes that kill pathogenic cells. Discover the latest research on the Alternative Complement Pathway.