Turner’s Syndrome, 46X, del (X) (p 11), Persistent Complement Activation and Membranoproliferative Glomerulonephritis

1982 ◽  
Vol 2 (5) ◽  
pp. 272-275 ◽  
Author(s):  
Paul R. Goodyer ◽  
Jack S.C. Fong ◽  
Bernard S. Kaplan
Keyword(s):  
Complement Activation ◽  
Membranoproliferative Glomerulonephritis

Morphofunctional Effects of C5 Convertase Blockade in Immune Complex-Mediated Membranoproliferative Glomerulonephritis: Report of Two Cases with Evidence of Terminal Complement Activation

Nephron ◽  
2020 ◽  
Vol 144 (4) ◽  
pp. 195-203
Author(s):  
Camillo Carrara ◽  
Manuel Alfredo Podestà ◽  
Mauro Abbate ◽  
Paola Rizzo ◽  
Rossella Piras ◽  
...  
Keyword(s):  
Immune Complex ◽  
Complement Activation ◽  
Membranoproliferative Glomerulonephritis ◽  
Terminal Complement

Thrombotic microangiopathy and smoldering multiple myeloma after kidney transplant in a patient with MCP mutation

2021 ◽  
pp. 239936932110626
Author(s):  
Marina Almenara Tejederas ◽  
Laura De la Torre Corona ◽  
Fabiola Alonso García ◽  
María Ángeles Rodríguez Pérez ◽  
Rocío Cabrera Pérez ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Renal Biopsy ◽  
Kidney Transplant ◽  
Complement Activation ◽  
Thrombotic Microangiopathy ◽  
Membranoproliferative Glomerulonephritis ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Membrane Cofactor Protein ◽  
Susceptible Patient ◽  
Uremic Syndrome

The most frequent cause of atypical hemolytic uremic syndrome (aHUS) is defective regulation of complement activation because of genetic anomalies. We present the case of 53-year-old man with a kidney transplant and stabilized kidney function (creatinine 2.5 mg/dL; proteinuria 0.4 g/24 h) with mycophenolate/tacrolimus/prednisone who was diagnosed of Thrombotic Microangiopathy (TMA). This diagnosis was associated with creatinine and proteinuria rise (3 mg/dL; 2.4 g/24 h) and a new monoclonal IgA/lambda component. Renal biopsy showed membranoproliferative glomerulonephritis; a pathogenic variant in the Membrane cofactor protein (MCP) gene with a polymorphism ggaac, typically associated to secondary aHUS, was identified. We suspected that immunoglobulin could be acting as a trigger for TMA in a genetically susceptible patient, so “clone-directed” therapy with bortezomib and dexamethasone was initiated.

scholarly journals Factor D Inhibition Blocks Complement Activation Induced by Mutant Factor B Associated With Atypical Hemolytic Uremic Syndrome and Membranoproliferative Glomerulonephritis

2021 ◽  
Vol 12 ◽  
Author(s):  
Sigridur Sunna Aradottir ◽  
Ann-Charlotte Kristoffersson ◽  
Lubka T. Roumenina ◽  
Anna Bjerre ◽  
Pavlos Kashioulis ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Hemolytic Uremic Syndrome ◽  
Complement Activation ◽  
Membranoproliferative Glomerulonephritis ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Complement Factor ◽  
Gain Of Function ◽  
Factor B ◽  
Glomerular Endothelial Cells ◽  
Uremic Syndrome

Complement factor B (FB) mutant variants are associated with excessive complement activation in kidney diseases such as atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy and membranoproliferative glomerulonephritis (MPGN). Patients with aHUS are currently treated with eculizumab while there is no specific treatment for other complement-mediated renal diseases. In this study the phenotype of three FB missense variants, detected in patients with aHUS (D371G and E601K) and MPGN (I242L), was investigated. Patient sera with the D371G and I242L mutations induced hemolysis of sheep erythrocytes. Mutagenesis was performed to study the effect of factor D (FD) inhibition on C3 convertase-induced FB cleavage, complement-mediated hemolysis, and the release of soluble C5b-9 from glomerular endothelial cells. The FD inhibitor danicopan abrogated C3 convertase-associated FB cleavage to the Bb fragment in patient serum, and of the FB constructs, D371G, E601K, I242L, the gain-of-function mutation D279G, and the wild-type construct, in FB-depleted serum. Furthermore, the FD-inhibitor blocked hemolysis induced by the D371G and D279G gain-of-function mutants. In FB-depleted serum the D371G and D279G mutants induced release of C5b-9 from glomerular endothelial cells that was reduced by the FD-inhibitor. These results suggest that FD inhibition can effectively block complement overactivation induced by FB gain-of-function mutations.

scholarly journals In situ complement activation in porcine membranoproliferative glomerulonephritis type II

1998 ◽  
Vol 53 (2) ◽  
pp. 331-349 ◽  
Author(s):  
Johan H. Jansen ◽  
Kolbjørn Høgåsen ◽  
Morten Harboe ◽  
Torstein Hovig
Keyword(s):  
Complement Activation ◽  
Membranoproliferative Glomerulonephritis ◽  
Type Ii
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