Recurrent haemolytic uraemic syndrome in a boy with focal and segmental glomerulosclerosis

Bökenkamp, Arend; Hoyer, Peter F.; Offner, G.; Helmchen, U.; Brodehl, J.

doi:10.1007/bf01959094

Cited by 9 publications

(7 citation statements)

References 8 publications

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“…67 An MCP mutation was detected in a child with TMA following treatment with cisplatin and carboplatin. 68 Several glomerulopathies, such as SLE, 69 immunoglobulin A (IgA) nephropathy, 70 focal segmental glomerulosclerosis (FSGS), 71,72 membranous nephropathy (MN), 73 and membranoproliferative glomerulonephritis (MPGN) 74 as well as vasculitides, especially antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), 75 coexist with TMA.…”

Section: Pathophysiology Within the Thrombotic Microangiopathy Spectrmentioning

confidence: 99%

Spectrum of Complement-Mediated Thrombotic Microangiopathies: Pathogenetic Insights Identifying Novel Treatment Approaches

Riedl

Fakhouri

Quintrec

et al. 2014

Semin Thromb Hemost

125

116

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Thrombotic microangiopathy (TMA) is a rare but severe disorder characterized by endothelial cell activation and thrombus formation. It manifests with the triad of hemolytic anemia, thrombocytopenia, and organ failure. Prompt diagnosis and treatment initiation are crucial for long-term outcome. TMA often manifests subsequent to infectious events, of which (enterohemorrhagic) Escherichia coli is the most frequently reported. TMA also occurs on the background of genetic/autoimmune defects in the complement system (atypical hemolytic uremic syndrome [aHUS]) and underlying conditions, such as pregnancy, transplantation, drugs, other glomerulopathies, vasculitides, or metabolic defects. Complement activation or defects in its regulation have now been described in an increasing number of acquired diseases with TMA. Coinciding with this expanding spectrum of complement-mediated diseases, the question arises which patients might benefit from a complement-targeted therapy. Success of therapy depends on the individual contribution of complement activation in disease pathogenesis. The advent of eculizumab, a monoclonal antibody that blocks terminal complement activation, has markedly improved outcome and quality of life in patients with aHUS. This review discusses the contribution of complement and highlights its complex interaction with inflammation, coagulation, and the endothelium. Treatment experiences focusing on eculizumab therapy are discussed in detail across the emerging spectrum of complement-mediated thrombotic microangiopathies.

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Section: Pathophysiology Within the Thrombotic Microangiopathy Spectrmentioning

confidence: 99%

Spectrum of Complement-Mediated Thrombotic Microangiopathies: Pathogenetic Insights Identifying Novel Treatment Approaches

Riedl

Fakhouri

Quintrec

et al. 2014

Semin Thromb Hemost

125

116

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“…In our patient, there was a histopathological evidence of TMA involving intra-renal artery with C3 deposits. HUS is also described in association with other chronic glomerular diseases such as focal segmental glomerulosclerosis (FSGS),[ 2 ] membranous glomerulonephritis,[ 3 ] membrano-proliferative glomerulonephritis (MPGN). [ 13 ] Almost all the cases, which are described above, shared one feature, the nephrotic state at the onset of HUS.…”

Section: Discussionmentioning

confidence: 99%

“…Thrombotic microangiopathy (TMA) leading to dialysis-dependent renal failure is rare. [ 1 ] Although TMA secondary to glomerular diseases has been reported,[ 1 2 3 4 ] TMA with hemolytic uremic syndrome (HUS) and acute cortical necrosis (ACN) in association with IgAN has been rarely reported.…”

Section: Introductionmentioning

confidence: 99%

Plasma exchange in Immunoglobulin A nephropathy with thrombotic microangiopathy and acute cortical necrosis

Doddi¹,

Gowda²,

Ramachandran³

et al. 2016

Indian J Nephrol

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A 25-year-old female presented with decreased urine output, deranged renal function, thrombocytopenia, and hemolytic anemia. Kidney biopsy was consistent with thrombotic microangiopathy with acute cortical necrosis and Immunoglobulin A nephropathy (IgAN). Hemolytic anemia, thrombocytopenia and urine output improved after five sessions of plasma exchange. Renal function showed a delayed recovery and serum creatinine normalized by 3 months. This is first case of successful use of plasma exchange in hemolytic uremic syndrome with cortical necrosis associated with IgAN.

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“…(Manenti et al, 2013;Manenti et al, 2015) In up to 50% of patients with C3 glomerulopathy, mutations of, or antibodies to the alternative pathway complement components were found. (Barbour et al, 2016;Medjeral-Thomas et al, 2013;Pickering et al, 2013;Skerka et al, 2009) Complement abnormalities have also been described in patients with focal segmental glomerulosclerosis (Benz et al, 2007;Bokenkamp et al, 1992) and membranous nephropathy. Complement abnormalities were also detected in 86% of patients with pregnancy-associated HUS, (Fakhouri et al, 2010) in 43% of patients with cobalamin C deficiency, (Bouts et al, 2010;Geraghty et al, 1992;Komhoff et al, 2013) and in malignant hypertension.…”

Section: Complement Dysregulation Is Central To Thrombotic Microangiomentioning

confidence: 99%

Calcineurin inhibitor-induced endothelial cell injury – A role for complement

Teoh

Riedl

Bruno

et al. 2018

Molecular Immunology

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Calcineurin inhibitor (CNI) use significantly reduced acute rejection rates and revolutionized early allograft and patient outcomes post-transplantation. However, known nephro-and vascular toxicity preclude its use and contribute to chronic allograft injury, negatively impacting longer term outcomes. Evolving evidence suggest a role for complement dysregulation in the pathogenesis of CNI-induced endothelial injury. In an in vitro model using Blood Outgrowth Endothelial Cells (BOEC) isolated from healthy donors and a donor with a pathogenic MCP/CD46 variant, we showed that cyclosporine resulted in a dose and time dependent increase in complement deposition, enhanced by anti-CD59 sensitization. We showed for the first time that MCP/CD46 deficient BOECs were genetically predisposed to be more susceptible to cyclosporine-induced complement deposition (multiple-hit hypothesis). We found complement factor H surface dysregulation to play a key role in cyclosporine-induced complement activation on BOEC surfaces, with glycocalyx abolishment possibly being the key mechanism leading to alternative pathway dysregulation.iii

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Recurrent haemolytic uraemic syndrome in a boy with focal and segmental glomerulosclerosis

Abstract: Pediatrics 9 Springer-Verlag 1992 R e c u r r e n t h a e m o l y t i c uraemic s y n d r o m e in a b o y with focal and s e g m e n t a l g l o m e r u l o s c l e r o s i

Cited by 9 publications

References 8 publications

Spectrum of Complement-Mediated Thrombotic Microangiopathies: Pathogenetic Insights Identifying Novel Treatment Approaches

Spectrum of Complement-Mediated Thrombotic Microangiopathies: Pathogenetic Insights Identifying Novel Treatment Approaches

Plasma exchange in Immunoglobulin A nephropathy with thrombotic microangiopathy and acute cortical necrosis

Calcineurin inhibitor-induced endothelial cell injury – A role for complement

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