Results 71 to 80 of about 24,341 (292)

Heterogeneity but individual constancy of epitopes, isotypes and avidity of factor H autoantibodies in atypical hemolytic uremic syndrome [PDF]

, 2016
Factor H (FH) autoantibodies are present in 6-10% of atypical hemolytic uremic syndrome (aHUS) patients, most of whom have homozygous deficiency of the FH-related protein FHR-1. Although the pathogenic role of the autoantibodies is established, little is
Bernabeu-Herrero ME,, Hyvarinen S,, Jokiranta TS, Józsi, Mihály, Lopez-Trascasa M, Nozal P,, Prohászka, Zoltán, Sanchez-Corral P, Szilágyi, Ágnes, Uzonyi, Barbara   +9 more
core   +2 more sources

Factor H autoantibody is associated with atypical hemolytic uremic syndrome in children in the United Kingdom and Ireland [PDF]

, 2017
Factor H autoantibodies can impair complement regulation, resulting in atypical hemolytic uremic syndrome, predominantly in childhood. There are no trials investigating treatment, and clinical practice is only informed by retrospective cohort ...
Almond, M, Awan, A, Booth, C, Brocklebank, V, Finlay, E, Fitzpatrick, M, Gilbert, RD, Goodship, TH, Hegde, S, Inward, C, Johnson, S, Kaur, A, Kavanagh, D, Kerecuk, L, Kinoshita, M, Marchbank, KJ, Marks, SD, Salama, AD, Sheerin, NS, Sheerin, TP, Tyerman, K, Walsh, PR, Webb, N, Wong, EKS   +23 more
core   +1 more source

Expert Statements on the Standard of Care in Critically Ill Adult Patients With Atypical Hemolytic Uremic Syndrome. [PDF]

, 2017
A typical hemolytic uremic syndrome (aHUS) presents similarly to thrombotic thrombocytopenic purpura (TTP) and other causes or conditions with thrombotic microangiopathy (TMA), such as disseminated intravascular coagulation or sepsis.
Abroug, F., Azoulay, E., Benoit, D., Eggimann, P., Galstian, G., Garnacho-Montero, J., Knoebl, P., Rusinova, K., Scully, M., von Bergwelt-Baildon, M., Wendon, J.   +10 more
core   +3 more sources

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

, 2008
Atypical hemolytic uremic syndrome (aHUS) is a severe renal disease that is associated with defective complement regulation caused by multiple factors.
Józsi, Mihály, Licht, C., Richter, H., Strobel, S., Zipfel, S. L.   +4 more
core   +1 more source

Serological and genetic complement alterations in infection-induced and complement-mediated hemolytic uremic syndrome [PDF]

, 2016
Background: The role of complement in the atypical form of hemolytic uremic syndrome (aHUS) has been investigated extensively in recent years. As the HUS-associated bacteria Shiga-toxin-producing Escherichia coli (STEC) can evade the complement system ...
Bakker, J.A. (Jaap), Bouts, A.H. (Antonia), Dorresteijn, E.M. (Eiske), Goertz, J. (Joop), Gracchi, V. (Valentina), Heuvel, L.P. (Lambert) van den, Jeronimus-Klaasen, A. (Annelies), Kar, N.C. (Nicole) van de, Keijzer-Veen, M.G. (Mandy), Roos, A. (Anja), van der Molen, R.G. (Renate G.), van der Velden, T.J.A.M. (Thea J. A. M.), van Wijk, J.A.E. (Joanna A. E.), Volokhina, E.B. (Elena), Westra, D.F.   +14 more
core   +6 more sources

Comparative efficacy of ravulizumab and eculizumab in the treatment of atypical hemolytic uremic syndrome: An indirect comparison using clinical trial data

Clinical Nephrology, 2021
Ravulizumab and eculizumab are approved terminal complement inhibitor treatments for atypical hemolytic uremic syndrome (aHUS). Ravulizumab was engineered from eculizumab to have an increased half-life allowing for reduced dosing frequency (8-weekly vs ...
I. Tomazos, Anthony J. Hatswell, S. Cataland, Peter Chen, N. Freemantle, Åsa Lommelé, K. Deighton, Emma S. Knowles, N. Sheerin, E. Rondeau   +9 more
semanticscholar   +1 more source

Genital Crohn's disease in pediatrics and genetic associations

JPGN Reports, EarlyView.
Abstract Genital edema is a rare presentation of Crohn's disease (CD), also known as metastatic CD (MCD). This may precede, co‐occur with, or follow gastrointestinal symptoms and present a diagnostic challenge. We aimed to characterize the features, clinical courses, pathogenesis, and outcomes of patients with MCD to increase understanding and promote ...
Erica Chang, Caroline Chinchilla Putzeys, Edward Hoffenberg, Ashish Patel, Elizabeth Hilow, Brad Pasternak   +5 more
wiley   +1 more source

On the relevance of thrombomodulin variants in atypical hemolytic uremic syndrome

Kidney International, 2023
5 p.-1 fig.-1 tab.
Ana Huerta, Emi Arjona, Jose Portoles, Paula Lopez-Sanchez, Teresa Cavero, Jeanette Fernandez-Cusicanqui, Miquel Blasco, Virginia Cabello, Natividad Calvo, Montserrat Diaz, Maria Herrero-Goñi, Mireia Aguirre, Sandra Elías, Maria Paz Alcaide, Natalia Ramos, Joana Sellares, Santiago Rodríguez de Córdoba   +16 more
openaire   +5 more sources

The global aHUS registry: methodology and initial patient characteristics [PDF]

, 2015
Background: Atypical hemolytic uremic syndrome (aHUS) is a rare, genetically-mediated systemic disease most often caused by chronic, uncontrolled complement activation that leads to systemic thrombotic microangiopathy (TMA) and renal and other end-organ ...
Ardissino, Gianluigi, Ariceta, Gema, Cohen, David, Cole, J. Alexander, Frémeaux-Bacchi, Véronique, Gasteyger, Christoph, Greenbaum, Larry A., Johnson, Sally, Licht, Christoph, Ogawa, Masayo, Schaefer, Franz, Vande Walle, Johan   +11 more
core   +2 more sources

COVID-19-associated atypical hemolytic uremic syndrome and use of Eculizumab therapy

JN. Journal of Nephrology (Milano. 1992), 2021
There is a high incidence of acute kidney injury with COVID-19 infections. The etiologies of acute kidney injury could be ischemic acute tubular necrosis or a complex process of complement activation leading to thrombotic microangiopathy.
J. Gill, C. Hebert, Gates B Colbert
semanticscholar   +1 more source