aHUS caused by complement dysregulation: new therapies on the horizon (Q33390198)

26 July 2017

5 references

27 September 2019

27 November 2019

13 December 2019

14 October 2020

29 October 2020

aHUS caused by complement dysregulation: new therapies on the horizon (English)

1 reference

complement system proteins

26 July 2017

1 reference

27 September 2019

1 reference

27 November 2019

1 reference

13 December 2019

1 reference

14 October 2020

1 reference

29 October 2020

corrigendum / erratum

Erratum to: aHUS caused by complement dysregulation: new therapies on the horizon.

0 references

2

Christoph Licht

1 reference

26 July 2017

Aoife M Waters

1

1 reference

26 July 2017

publication date

18 June 2010

1 reference

26 July 2017

1 January 2011

5 references

27 September 2019

27 November 2019

13 December 2019

14 October 2020

29 October 2020

6 references

26 July 2017

27 September 2019

27 November 2019

13 December 2019

14 October 2020

29 October 2020

26

1 reference

26 July 2017

issue

1

1 reference

26 July 2017

page(s)

41-57

1 reference

Mutations in components of complement influence the outcome of Factor I-associated atypical hemolytic uremic syndrome

26 July 2017

cites work

1 reference

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Therapeutic potential of complement modulation

21 June 2018

1 reference

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Successful renal transplantation in factor H autoantibody associated HUS with CFHR1 and 3 deficiency and CFH variant G2850T.

21 June 2018

1 reference

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Mutations in complement factor I as found in atypical hemolytic uremic syndrome lead to either altered secretion or altered function of factor I

21 June 2018

1 reference

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Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome

21 June 2018

1 reference

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Severe atypical HUS caused by CFH S1191L--case presentation and review of treatment options

21 June 2018

1 reference

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Maintenance of kidney function following treatment with eculizumab and discontinuation of plasma exchange after a third kidney transplant for atypical hemolytic uremic syndrome associated with a CFH mutation

21 June 2018

1 reference

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Atypical hemolytic-uremic syndrome

21 June 2018

1 reference

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Clinical practice guidelines for the management of atypical haemolytic uraemic syndrome in the United Kingdom

21 June 2018

1 reference

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Characterization of complement factor H-related (CFHR) proteins in plasma reveals novel genetic variations of CFHR1 associated with atypical hemolytic uremic syndrome

21 June 2018

1 reference

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Complement regulators and inhibitory proteins

21 June 2018

1 reference

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Clinical practice. Today's understanding of the haemolytic uraemic syndrome

21 June 2018

1 reference

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Functional analyses indicate a pathogenic role of factor H autoantibodies in atypical haemolytic uraemic syndrome

21 June 2018

1 reference

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Thrombomodulin mutations in atypical hemolytic-uremic syndrome

21 June 2018

1 reference

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A large family with a gain-of-function mutation of complement C3 predisposing to atypical hemolytic uremic syndrome, microhematuria, hypertension and chronic renal failure

21 June 2018

1 reference

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Hemolytic uremic syndrome due to homozygous factor H deficiency

21 June 2018

1 reference

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Complement inhibitor eculizumab in atypical hemolytic uremic syndrome

21 June 2018

1 reference

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Atypical hemolytic uremic syndrome associated with complement factor H autoantibodies and CFHR1/CFHR3 deficiency

21 June 2018

1 reference

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Successful renal transplantation in a patient with atypical hemolytic uremic syndrome carrying mutations in both factor I and MCP.

21 June 2018

1 reference

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Anti-Factor H autoantibodies in a fifth renal transplant recipient with atypical hemolytic and uremic syndrome.

21 June 2018

1 reference

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Autoantibodies in haemolytic uraemic syndrome (HUS).

21 June 2018

1 reference

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Liver-kidney transplantation to cure atypical hemolytic uremic syndrome

21 June 2018

1 reference

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Successful split liver-kidney transplant for factor H associated hemolytic uremic syndrome

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Recurrent atypical hemolytic uremic syndrome associated with factor I mutation in a living related renal transplant recipient

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

FDA report: eculizumab (Soliris) for the treatment of patients with paroxysmal nocturnal hemoglobinuria

21 June 2018

1 reference

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Complement and the atypical hemolytic uremic syndrome in children.

21 June 2018

1 reference

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Inherited deficiency of membrane cofactor protein expression and varying manifestations of recurrent atypical hemolytic uremic syndrome in a sibling pair

21 June 2018

1 reference

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Hemolytic uremic syndrome recurrence after renal transplantation

21 June 2018

1 reference

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Efficacy of plasma therapy in atypical hemolytic uremic syndrome with complement factor H mutations

21 June 2018

1 reference

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Complement factor H deficiency and posttransplantation glomerulonephritis with isolated C3 deposits

21 June 2018

1 reference

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Successful pre-transplant management of a patient with anti-factor H autoantibodies-associated haemolytic uraemic syndrome

21 June 2018

1 reference

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Translational mini-review series on complement factor H: renal diseases associated with complement factor H: novel insights from humans and animals.

21 June 2018

1 reference

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Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/CFHR3 deficiency

21 June 2018

1 reference

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Discovery and development of the complement inhibitor eculizumab for the treatment of paroxysmal nocturnal hemoglobinuria

21 June 2018

1 reference

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Successful liver-kidney transplantation in two children with aHUS caused by a mutation in complement factor H.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Complement evasion of pathogens: common strategies are shared by diverse organisms

21 June 2018

1 reference

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Outcome of renal transplantation in patients with non-Shiga toxin-associated hemolytic uremic syndrome: prognostic significance of genetic background

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Recurrence of HUS due to CD46/MCP mutation after renal transplantation: a role for endothelial microchimerism.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Differential impact of complement mutations on clinical characteristics in atypical hemolytic uremic syndrome

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Characterization of mutations in complement factor I (CFI) associated with hemolytic uremic syndrome

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Anti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome

21 June 2018

1 reference

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Complete factor H deficiency-associated atypical hemolytic uremic syndrome in a neonate

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome

21 June 2018

1 reference

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A missense mutation in factor I (IF) predisposes to atypical haemolytic uraemic syndrome

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Thrombin-activatable procarboxypeptidase B regulates activated complement C5a in vivo

21 June 2018

1 reference

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A mutation in factor I that is associated with atypical hemolytic uremic syndrome does not affect the function of factor I in complement regulation

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Atypical haemolytic uraemic syndrome associated with a hybrid complement gene

21 June 2018

1 reference

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Atypical haemolytic uraemic syndrome

21 June 2018

1 reference

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Genetic abnormalities of complement regulators in hemolytic uremic syndrome: how do they affect patient management?

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Secondary failure of plasma therapy in factor H deficiency

21 June 2018

1 reference

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Successful prophylactic plasma infusions in recurrent atypical hemolytic-uremic syndrome

21 June 2018

1 reference

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Bacterial complement evasion.

21 June 2018

1 reference

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A classification of hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and related disorders

21 June 2018

1 reference

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Genetic and functional analyses of membrane cofactor protein (CD46) mutations in atypical hemolytic uremic syndrome

21 June 2018

1 reference

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Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome

21 June 2018

1 reference

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Phenotypic expression of factor H mutations in patients with atypical hemolytic uremic syndrome.

21 June 2018

1 reference

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Complement factor H-associated atypical hemolytic uremic syndrome in monozygotic twins: concordant presentation, discordant response to treatment

21 June 2018

1 reference

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Role of complement and other innate immune mechanisms in the removal of apoptotic cells

21 June 2018

1 reference

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Insights into hemolytic uremic syndrome: segregation of three independent predisposition factors in a large, multiple affected pedigree

21 June 2018

1 reference

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An interactive web database of factor H-associated hemolytic uremic syndrome mutations: insights into the structural consequences of disease-associated mutations

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome

21 June 2018

1 reference

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Hemolytic uremic syndrome: a fatal outcome after kidney and liver transplantation performed to correct factor h gene mutation

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Successful plasma therapy for atypical hemolytic uremic syndrome caused by factor H deficiency owing to a novel mutation in the complement cofactor protein domain 15.

21 June 2018

1 reference

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Predisposition to atypical hemolytic uremic syndrome involves the concurrence of different susceptibility alleles in the regulators of complement activation gene cluster in 1q32.

21 June 2018

1 reference

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Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome

21 June 2018

1 reference

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Challenges in the management of infantile factor H associated hemolytic uremic syndrome.

21 June 2018

1 reference

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Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome

21 June 2018

1 reference

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Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Familial haemolytic uraemic syndrome and an MCP mutation

21 June 2018

1 reference

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Complement factor H mutations and gene polymorphisms in haemolytic uraemic syndrome: the C-257T, the A2089G and the G2881T polymorphisms are strongly associated with the disease

21 June 2018

1 reference

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Mutations in human complement regulator, membrane cofactor protein (CD46), predispose to development of familial hemolytic uremic syndrome

21 June 2018

1 reference

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Successful (?) therapy of hemolytic-uremic syndrome with factor H abnormality

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Mutations in factor H reduce binding affinity to C3b and heparin and surface attachment to endothelial cells in hemolytic uremic syndrome

21 June 2018

1 reference

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Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Recurrence of hemolytic uremic syndrome after live related renal transplantation associated with subsequent de novo disease in the donor

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Therapeutic plasma exchange and plasma infusion in thrombotic microvascular syndromes

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Plasmapheresis in thrombotic microangiopathy-associated syndromes: review of outcome data derived from clinical trials and open studies.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Combined kidney and liver transplantation for familial haemolytic uraemic syndrome

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Decoding the patterns of self and nonself by the innate immune system

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Successful plasma therapy in hemolytic uremic syndrome with factor H deficiency.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Familial hemolytic uremic syndrome associated with complement factor H deficiency

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Clustering of missense mutations in the C-terminal region of factor H in atypical hemolytic uremic syndrome.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Complications of plasma exchange in 71 consecutive patients treated for clinically suspected thrombotic thrombocytopenic purpura-hemolytic-uremic syndrome

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

FHL-1/reconectin: a human complement and immune regulator with cell-adhesive function

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Human complement factor H deficiency associated with hemolytic uremic syndrome.

21 June 2018

1 reference

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Hypocomplementemic autosomal recessive hemolytic uremic syndrome with decreased factor H.

21 June 2018

1 reference

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Genetic studies into inherited and sporadic hemolytic uremic syndrome

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Familial hemolytic-uremic syndrome and homozygous factor H deficiency

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2991208

Inherited factor H deficiency and collagen type III glomerulopathy

21 June 2018

1 reference

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Haemolytic uraemic syndromes in the British Isles, 1985-8: association with verocytotoxin producing Escherichia coli. Part 2: Microbiological aspects

21 June 2018

1 reference

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Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients

21 June 2018

1 reference

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Cobalamin C defect associated with hemolytic-uremic syndrome

21 June 2018

1 reference

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Hemolytic Uremic Syndrome in Families

21 June 2018

1 reference

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Control of the amplification convertase of complement by the plasma protein beta1H

21 June 2018

1 reference

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Hemolytic uremic syndrome with recurrent episodes: an important subset

21 June 2018

1 reference

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Human complement C3b inactivator: isolation, characterization, and demonstration of an absolute requirement for the serum protein beta1H for cleavage of C3b and C4b in solution

21 June 2018

1 reference

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Haemolytic-uraemic syndrome: deficiency of plasma factor(s) regulating prostacyclin activity?

21 June 2018

1 reference

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Modulation of the alternative complement pathways by beta 1 H globulin

21 June 2018

1 reference

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Eculizumab for congenital atypical hemolytic-uremic syndrome.

21 June 2018

1 reference

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Eculizumab for paroxysmal nocturnal haemoglobinuria

31 October 2018

1 reference

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Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome.

31 October 2018

1 reference

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Effect of eculizumab on hemolysis and transfusion requirements in patients with paroxysmal nocturnal hemoglobinuria

31 October 2018

1 reference

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Heterozygous and homozygous factor H deficiency states in a Dutch family

31 October 2018

1 reference

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Eculizumab for Atypical Hemolytic–Uremic Syndrome

31 October 2018

1 reference

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Guidelines for the use of fresh-frozen plasma, cryoprecipitate and cryosupernatant

31 October 2018

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Successful treatment of factor H-related haemolytic uraemic syndrome

10 December 2018

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12 December 2020

Combined homozygous factor H and heterozygous C2 deficiency in an Italian family

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12 December 2020

Hereditary, complete deficiency of complement factor H associated with recurrent meningococcal disease

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12 December 2020

Hypocomplementaemia due to a genetic deficiency of beta 1H globulin

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12 December 2020

Factor H deficiency and fibrillary glomerulopathy

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12 December 2020

ESCHERICHIA COLI CYTOTOXIN, HAEMOLYTIC-URAEMIC SYNDROME, AND HAEMORRHAGIC COLITIS

1 reference

12 December 2020

H deficiency in two brothers with atypical dense intramembranous deposit disease

1 reference

12 December 2020

1 reference

12 December 2020

Molecular characterization of Complement Factor I deficiency in two Spanish families

1 reference

12 December 2020

1 reference

12 December 2020

Safety and long-term efficacy of eculizumab in a renal transplant patient with recurrent atypical hemolytic-uremic syndrome

1 reference

12 December 2020

10.1007/S00467-010-1556-4

Identifiers

DOI

1 reference

Dimensions Publication ID

26 July 2017

0 references

1 reference

26 July 2017

1 reference