scholarly journals A 'silent', new polymorphism of factor H and apparent de novo atypical haemolytic uraemic syndrome after kidney transplantation

2014 ◽  
Vol 2014 (dec23 1) ◽  
pp. bcr2014207630-bcr2014207630 ◽  
Author(s):  
E. N. Broeders ◽  
P. Stordeur ◽  
S. Rorive ◽  
K. Dahan
Keyword(s):  
Kidney Transplantation ◽  
Haemolytic Uraemic Syndrome ◽  
De Novo ◽  
Factor H ◽  
Atypical Haemolytic Uraemic Syndrome

scholarly journals SP837ECULIZUMAB TREATMENT IN DE NOVO ATYPICAL HAEMOLYTIC URAEMIC SYNDROME AFTER RENAL TRANSPLANTATION ASSOCIATED WITH COMPLETE DEFICIENCY OF FACTOR-H RELATED PROTEINS 1 AND 3

2015 ◽  
Vol 30 (suppl_3) ◽  
pp. iii653-iii654
Author(s):  
Elena Román-Ortiz ◽  
Santiago Mendizábal ◽  
Jaouad Anter ◽  
Margarita López-Trascasa ◽  
Pilar Sánchez-Corral ◽  
...  
Keyword(s):  
Renal Transplantation ◽  
Haemolytic Uraemic Syndrome ◽  
De Novo ◽  
Factor H ◽  
Atypical Haemolytic Uraemic Syndrome ◽  
Related Proteins

scholarly journals De Novo Atypical Haemolytic Uremic Syndrome after Kidney Transplantation

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
Arnaud Devresse ◽  
Martine de Meyer ◽  
Selda Aydin ◽  
Karin Dahan ◽  
Nada Kanaan
Keyword(s):  
Kidney Transplantation ◽  
De Novo ◽  
Alternative Pathway ◽  
Factor H ◽  
Complement Factor ◽  
Haemolytic Uremic Syndrome ◽  
Hinman Syndrome ◽  
Uremic Syndrome ◽  
Stage Renal Disease ◽  
End Stage

De novo thrombotic microangiopathy (TMA) can occur after kidney transplantation. An abnormality of the alternative pathway of complement must be suspected and searched for, even in presence of a secondary cause. We report the case of a 23-year-old female patient who was transplanted with a kidney from her mother for end-stage renal disease secondary to Hinman syndrome. Early after transplantation, she presented with 2 episodes of severe pyelonephritis, associated with acute kidney dysfunction and biological and histological features of TMA. Investigations of the alternative pathway of the complement system revealed atypical haemolytic uremic syndrome secondary to complement factor I mutation, associated with mutations in CD46 and complement factor H related protein genes. Plasma exchanges followed by eculizumab injections allowed improvement of kidney function without, however, normalization of creatinine.

scholarly journals Novel complement factor H gene mutation causing atypical haemolytic uraemic syndrome: early Eculizumab prevents acute dialysis

2017 ◽  
Vol 10 (2) ◽  
pp. 263-265
Author(s):  
James Collett ◽  
Amali Mallawaarachchi ◽  
Eddy Fischer ◽  
Muralikrishna Gangadharan Komala ◽  
Kamal Sud ◽  
...  
Keyword(s):  
Gene Mutation ◽  
Haemolytic Uraemic Syndrome ◽  
Factor H ◽  
Complement Factor H ◽  
Complement Factor ◽  
Acute Dialysis ◽  
Atypical Haemolytic Uraemic Syndrome ◽  
H Gene

scholarly journals Characteristics, management and outcomes of atypical haemolytic uraemic syndrome in kidney transplant patients: a retrospective national study

2020 ◽  
Author(s):  
José Portoles ◽  
Ana Huerta ◽  
Emilia Arjona ◽  
Eva Gavela ◽  
Marisa Agüera ◽  
...  
Keyword(s):  
High Risk ◽  
Haemolytic Uraemic Syndrome ◽  
De Novo ◽  
Pathogenic Mutation ◽  
National Study ◽  
Moderate Risk ◽  
Risk Of Recurrence ◽  
Transplant Patients ◽  
Atypical Haemolytic Uraemic Syndrome ◽  
Late Group

Abstract Background Kidney transplantation (KTx) is a strong trigger for the development of either recurrent or de novo atypical haemolytic uraemic syndrome (aHUS). According to previous studies, eculizumab (ECU) is effective for prophylaxis and for treatment of recurrence. Methods We evaluated the experiences of Spanish patients with recurrent and de novo aHUS associated with KTx, treated or not treated with ECU. In the de novo group, we classified patients as having early de novo (during the first month) or late de novo aHUS (subsequent onset). Results We analysed 36 cases of aHUS associated with KTx. All of the 14 patients with pre-KTx diagnosis of aHUS were considered to have high or moderate risk of recurrence. Despite receiving grafts from suboptimal donors, prophylactic ECU was effective for avoiding recurrence. The drug was stopped only in two cases with low–moderate risk of recurrence and was maintained in high-risk patients with no single relapse. There were 22 de novo aHUS cases and 16 belonged to the early de novo group. The median time of onset in the late group was 3.4 years. The early group had a better response to ECU than the late group, probably due to earlier diagnosis and use of the drug. No genetic pathogenic variant was detected in de novo aHUS cases, suggesting a secondary profile of the disease. ECU was stopped in all de novo patients with no relapses. ECU was well tolerated in all cases. Conclusions Both groups (pre-aHUS and de novo) presented different clinical profiles, management approaches and outcomes. One should consider aHUS regardless of time after KTx. Genetic studies are crucial to stratify risks of relapse and to determine necessary lengths of treatment. We suggest short ECU treatment for de novo cases without pathogenic mutation and that ECU treatment be considered pre-emptively for patients with moderate or high risk of recurrence.

The binding of factor H to a complex of the physiological polyanion and C3b on the cell surface is disrupted in atypical haemolytic uraemic syndrome

2008 ◽  
Vol 45 (16) ◽  
pp. 4099
Author(s):  
Viviana Ferreira ◽  
Andrew Herbert ◽  
Claudio Cortes ◽  
Kristi Mckee ◽  
Bärbel Blaum ◽  
...  
Keyword(s):  
Cell Surface ◽  
Haemolytic Uraemic Syndrome ◽  
Factor H ◽  
Atypical Haemolytic Uraemic Syndrome
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