Results 31 to 40 of about 24,893 (251)

Early Eculizumab Withdrawal in Patients With Atypical Hemolytic Uremic Syndrome in Native Kidneys Is Safe and Cost-Effective: Results of the CUREiHUS Study

Kidney International Reports, 2022
Introduction The introduction of eculizumab has improved the outcome in patients with atypical hemolytic uremic syndrome (aHUS). The optimal treatment strategy is debated.
R. Bouwmeester, Caroline Duineveld, K. Wijnsma, F. Bemelman, J. W. van der Heijden, Joanna A. E. van Wijk, A. Bouts, J. van de Wetering, E. Dorresteijn, S. Berger, Valentina Gracchi, A. V. van Zuilen, M. Keijzer-Veen, A. D. de Vries, R. W. van Rooij, F. Engels, Wim Altena, Renée de Wildt, Evy van Kempen, E. Adang, Mendy ter Avest, R. ter Heine, E. Volokhina, L. P. van den Heuvel, J. Wetzels, N. C. van de Kar   +25 more
semanticscholar   +1 more source

Influenza B-Associated Atypical Hemolytic Uremic Syndrome [PDF]

Revista Portuguesa de Nefrologia e Hipertensão, 2022
Hemolytic uremic syndrome (HUS) triggered by influenza virus (iHUS) is rare. Influenza A infections have been described to trigger atypical HUS (aHUS) in individuals with an underlying genetic complement dysregulation. To date there are only few reports
Lia Mano, Telma Francisco, Joana Gaspar, Gabriela Pereira, Raquel Santos, Margarida Abranches   +5 more
doaj   +1 more source

Atypical hemolytic uremic syndrome in the era of terminal complement inhibition: an observational cohort study

Blood, 2023
Key Points • We prove in genotype-matched complement-mediated aHUS cohorts that C5 inhibition results in a statistical improvement in ESKD-free survival.• We demonstrate that biallelic pathogenic mutations in EXOSC3 cause eculizumab nonresponsive aHUS.
V. Brocklebank, P. Walsh, K. Smith-Jackson, T. Hallam, K. Marchbank, V. Wilson, Theophile Bigirumurame, T. Dutt, Emma K Montgomery, M. Malina, E. K. Wong, Sally Johnson, N. Sheerin, D. Kavanagh, Richard Caroline Neil Stanley Suzanne Ravindra Declan Colm Holt Jones Ashman Fan Forbes Rajakariar De Freitas, Richard C. L. Holt, Caroline Jones, N. Ashman, Stanley Fan, Suzanne H Forbes, R. Rajakariar, Declan de Freitas, Colm Magee, Jennifer B. Hanko, David Milford, M. Muorah, A. Howie, Jyoti Baharani, Indranil Dasgupta, R. Roberts, A. MacDiarmaid-Gordon, A. Fry, N. Torpey, M. Waldron, Atif Awan, T. Raftery, Laura Ratcliffe, E. Vilar, Joel A. Newman, D. Seviar, Stephen D. Marks, L. Rees, Faidra Veligratli, Aoife Waters, Paramit Chowdhury, James M. Pattison, C. Booth, N. Williams, Richard Corbett, Beena Amin, Matthew C Pickering, K. Bramham, Mohamed Alhamed, A. Hill, Andrew Mooney, Eric Finlay, A. Newnham, Pallavi Yadav, Amrit Kaur, N. Plant, Mohan Shenoy, Lye Wai Choong, A. Helps, Robert McLemon, Thalakunte M Muniraju, Izhar H. Khan, D. Kidder, Neal Padmanabhan, Zoe Cousland, Jonathan Price, Alison Taylor, C. Jennings, Sohail Ahmad, Paul Mead, M. Aslam, Charlotte Bebb, C. Byrne, A. Ferraro, Martin Christian, Jonathan M. Evans, Jon Jin Kim, Andrew Lunn, M. Mallik, P. Mason, D. Mole, J. Craze, N. Sangala, Mark Uniacke, E. McGregor, S. Lambie, N. Bhandary, O. Flossmann, M. Mohteshamzadeh, A. Abeyaratne, Coralie Bingham, Rhian L. Clissold, L. Smyth, J. Connolly, B. Reynolds, J. Neary, Joanne Bell, S. Roxburgh, J. Popoola, Rosie Donne, Nicholas J M Fardon, Arif Khwaja, Shareen Siddiqi, G. Kumar, S. Kardasz, Iain Moore, D. Tez, Jamie Willows, R. Davison, John Harty, W. Wong, A. Williams, Daniel Gale, Richard J. Coward, C. Inward, Martin Mráz, Waqar Ayub, A. Short, David Lappin, Richard Baines, Girish Bommayya, K. Houlberg, I. Saif, Rodney Gilbert, Siân Griffin, Graham Smith, Judith van der Voort, J. Chandar, Michael Freundlich, F. McCarroll, Germaine Wong, P. Laboi, R. Molyneux   +136 more
semanticscholar   +1 more source

Pregnancy-Associated Atypical Hemolytic Uremic Syndrome: A Case Report with MCP Gene Mutation and Successful Eculizumab Treatment

American Journal of Perinatology Reports, 2023
Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare condition characterized by microangiopathic hemolytic anemia and kidney injury from thrombotic microangiopathy.
A. Domínguez-Vargas, Fanny Ariño, D. Silva, Henry J. González-Torres, Gustavo Aroca-Martínez, Eduardo Egea, C. G. Musso   +6 more
semanticscholar   +1 more source

Hyperfunctional complement C3 promotes C5-dependent atypical hemolytic uremic syndrome in mice [PDF]

, 2019
Atypical hemolytic uremic syndrome (aHUS) is frequently associated in humans with loss-of-function mutations in complement-regulating proteins or gain-of-function mutations in complement-activating proteins.
Atkinson, John P, Barlow, Paul N, Cook, H. T, Cooke, Katie, Denton, Harriet, Kavanagh, David, Liszewski, M. K, Marchbank, Kevin J, Pappworth, Isabel Y, Pickering, Matthew C, Smith-Jackson, Kate, Yang, Yi   +11 more
core   +3 more sources

The genetics of atypical hemolytic uremic syndrome [PDF]

Medizinische Genetik, 2018
Abstract Atypical hemolytic uremic syndrome (aHUS) is a disorder characterized by thrombocytopenia and microangiopathic hemolytic anemia due to endothelial injury. aHUS is felt to be caused by defective complement regulation due to underlying genetic mutations in complement regulators or activators, most often of the alternative pathway.
Feitz, W.JC., van de Kar, N.CA.J., Orth-Höller, D., van den Heuvel, L.PJ.W., Licht, C.   +4 more
openaire   +3 more sources

How I diagnose and treat atypical hemolytic uremic syndrome.

Blood, 2022
Our understanding and management of atypical haemolytic uremic syndrome (HUS) has dramatically improved in the last decade. Atypical HUS has been established as a prototypic disease resulting from a dysregulation of the complement alternative C3 ...
F. Fakhouri, Nora Schwotzer, V. Frémeaux-Bacchi   +2 more
semanticscholar   +1 more source

Analysis of linear antibody epitopes on factor H and CFHR1 using sera of patients with autoimmune atypical hemolytic uremic syndrome [PDF]

, 2017
Introduction: In autoimmune atypical hemolytic uremic syndrome (aHUS), the complement regulator factor H (FH) is blocked by FH autoantibodies, while 90% of the patients carry a homozygous deletion of its homolog complement FH-related protein 1 (CFHR1 ...
Csuka, Dorottya, Józsi, Mihály, Kelen, Kata, Prohászka, Zoltán, Reusz, György, Szabó, Attila, Szilágyi, Ágnes, Trojnár, Eszter, Uray, Katalin   +8 more
core   +5 more sources

Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors : a single-institution experience [PDF]

, 2010
Thrombotic microangiopathies are rare conditions characterized by microangiopathic hemolytic anemia, microthrombi, and multiorgan insult. The disorders, which include hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, are often acute and ...
Catlow, Jamie, Douglas, Kenneth W., Green, Rachel, Pollock, Kevin G.J., Young, David   +4 more
core   +1 more source

Eculizumab Versus Ravulizumab for the Treatment of Atypical Hemolytic Uremic Syndrome: A Systematic Review

Cureus, 2023
Atypical hemolytic uremic syndrome (aHUS) is a type of thrombotic microangiopathy and is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney failure. The complement cascade plays an integral role in aHUS.
Kamran Shahid, S. Qayyum
semanticscholar   +1 more source