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Anti-factor B autoantibody in dense deposit disease [PDF]
Dense deposit disease (DDD), also known as membranoproliferative glomerulonephritis type II, is a rare kidney disorder that is associated with dysregulation of the alternative pathway of complement.
Józsi, Mihály +4 more
core +7 more sources
Dense deposit disease (DDD) is an orphan disease that primarily affects children and young adults without sexual predilection. Studies of its pathophysiology have shown conclusively that it is caused by fluid-phase dysregulation of the alternative pathway of complement, however the role played by genetics and autoantibodies like C3 nephritic factors ...
Smith RJ, Harris CL, Pickering MC.
europepmc +6 more sources
Dense deposit disease and C3 glomerulopathy.
C3 glomerulopathy refers to those renal lesions characterized histologically by predominant C3 accumulation within the glomerulus, and pathogenetically by aberrant regulation of the alternative pathway of complement. Dense deposit disease is distinguished from other forms of C3 glomerulopathy by its characteristic appearance on electron microscopy. The
Barbour TD +2 more
europepmc +5 more sources
Eculizumab for dense deposit disease and C3 glomerulonephritis. [PDF]
Summary Background and objectives The principle defect in dense deposit disease and C3 glomerulonephritis is hyperactivity of the alternative complement pathway. Eculizumab, a monoclonal antibody that binds to C5 to prevent formation of the membrane attack complex, may prove beneficial.
Bomback AS +11 more
europepmc +6 more sources
Dense deposit disease is not a membranoproliferative glomerulonephritis [PDF]
Dense deposit disease (first reported in 1962) was classified as subtype II of membranoproliferative glomerulonephritis in the early 1970s. Over the last 30 years, marked differences in etiology and pathogenesis between type I membranoproliferative glomerulonephritis and dense deposit disease have become apparent.
Walker, Patrick D +3 more
openaire +5 more sources
Dense Deposit Disease Involving C3 and C4d Deposits. [PDF]
Dense deposit disease (DDD), earlier called Type II membranoproliferative glomerulonephritis is distinct disease having frequent relapses reaching end-stage kidney disease by 10-year in up to 50%-60% of cases and high recurrence rate in the allograft. The term DDD is derived from its distinctive ribbon-like osmiophilic deposits in the lamina densa of ...
Vankalakunti M +6 more
europepmc +3 more sources
Eculizumab in Pediatric Dense Deposit Disease. [PDF]
Dense deposit disease (DDD), a subtype of C3 glomerulopathy, is a rare disease affecting mostly children. Treatment options are limited. Debate exists whether eculizumab, a monoclonal antibody against complement factor C5, is effective in DDD. Reported data are scarce, especially in children.The authors analyzed clinical and histologic data of five ...
Oosterveld MJ +10 more
europepmc +5 more sources
Dense deposit disease complicated with choroidal neovascularization. [PDF]
Inanc M, Tekin K, Sekeroglu MA.
europepmc +3 more sources
A new complement factor B mutation associated with crescentic C3 glomerulopathy; a case report [PDF]
Background: C3 glomerulopathy is a recently described entity classified as complementassociated glomerular disease. Case Presentation: We report a case of a 48-year-old man referred to the nephrology department for nephrotic syndrome with rapidly ...
Sofia Semedo Coelho +10 more
doaj +1 more source
Pathology of C3 Glomerulopathy [PDF]
C3 glomerulopathy is a renal disorder involving dysregulation of alternative pathway complement activation. In most instances, a membranoproliferative pattern of glomerular injury with a prevalence of C3 deposition is observed by immunofluorescence ...
Su-Jin Shin, Yoonje Seong, Beom Jin Lim
doaj +1 more source

