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Atypical hemolytic uremic syndrome: diagnosis, management, and discontinuation of therapy. [PDF]

Hematology Am Soc Hematol Educ Program
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy typically characterized by anemia, thrombocytopenia, and end-organ injury. aHUS occurs due to endothelial injury resulting from overactivation of the alternative pathway of the ...
Java A.
europepmc   +4 more sources

Pediatric Atypical Hemolytic Uremic Syndrome Advances [PDF]

Cells, 2021
Atypical hemolytic uremic syndrome (aHUS) is a rare disorder characterized by dysregulation of the alternate pathway. The diagnosis of aHUS is one of exclusion, which complicates its early detection and corresponding intervention to mitigate its high ...
Rupesh Raina, Nina Vijayvargiya, Amrit Khooblall, Manasa Melachuri, Shweta Deshpande, Divya Sharma, Kashin Mathur, Manav Arora, Sidharth Kumar Sethi, Sonia Sandhu   +9 more
doaj   +4 more sources

The major autoantibody epitope on factor H in atypical hemolytic uremic syndrome is structurally different from its homologous site in factor H-related protein 1, supporting a novel model for induction of autoimmunity in this disease [PDF]

, 2015
Atypical hemolytic uremic syndrome (aHUS) is characterized by complement attack against host cells due to mutations in complement proteins or autoantibodies against complement factor H (CFH).
Abarrategui-Garrido, Adams, Adrian Goldman, Arnab Bhattacharjee, Baker, Barbara Uzonyi, Bhattacharjee, Bhattacharjee, Blanc, Blaum, Castiblanco-Valencia, Dragon-Durey, Dragon-Durey, Dragon-Durey, Emsley, Eszter Trojnár, Eyler, Ferreira, Freddo, Fritzler, Goicoechea de Jorge, Harald Seeberger, Hebecker, Heinen, Herbert, Herbert, Hofer, Holmes, Jokiranta, Józsi, Józsi, Józsi, Kabsch, Kajander, Lehtinen, Manuelian, McCarthy, McCoy, Meri, Mihály Józsi, Moore, Morgan, Noris, Oppermann, Pangburn, Robert Kolodziejczyk, Roumenina, Satu Hyvärinen, Sinha, Stefanie Reuter, Strobel, Strobel, Sánchez-Corral, Sánchez-Corral, T. Sakari Jokiranta, Venables, Zipfel, Zipfel, Zoltán Prohászka, Ágnes Szilágyi   +59 more
core   +3 more sources

A novel missense mutation in complement factor I predisposes patients to atypical hemolytic uremic syndrome: a case report

Journal of Medical Case Reports, 2022
Background Atypical hemolytic uremic syndrome, also called the nondiarrheal form of hemolytic uremic syndrome, is a rare disease characterized by the triad of thrombocytopenia, Coomb’s test-negative microangiopathic hemolytic anemia, and acute renal ...
Xin Wei, Juan Li, Xiaojiang Zhan, Luxia Tu, Haowen Huang, Ying Wang   +5 more
doaj   +2 more sources

Atypical hemolytic uremic syndrome : update on the complement system and what is new [PDF]

, 2010
Atypical hemolytic uremic syndrome (aHUS) is a rare disease of microangiopathic hemolytic anemia, thrombocytopenia, and predominant renal impairment. It is characterized by the absence of Shiga toxin-producing bacteria as a triggering factor.
Dickenmann, Michael, Hirt-Minkowski, Patricia, Schifferli, Jürg A.   +2 more
core   +3 more sources

Complement activation in atypical hemolytic uremic syndrome and scleroderma renal crisis: a critical analysis of pathophysiology

Brazilian Journal of Nephrology, 2018
Scleroderma is an autoimmune disease that affects multiple systems. While pathophysiologic mechanisms governing the development of scleroderma are relatively poorly understood, advances in our understanding of the complement system are clarifying the ...
Roman Zuckerman, Arif Asif, Eric J. Costanzo, Tushar Vachharajani   +3 more
doaj   +2 more sources

Use of Highly Individualized Complement Blockade Has Revolutionized Clinical Outcomes after Kidney Transplantation and Renal Epidemiology of Atypical Hemolytic Uremic Syndrome. [PDF]

Journal of the American Society of Nephrology, 2019
BACKGROUND Atypical hemolytic uremic syndrome (HUS) is associated with high recurrence rates after kidney transplant, with devastating outcomes. In late 2011, experts in France recommended the use of highly individualized complement blockade-based ...
J. Zuber, Marie Frimat, S. Caillard, N. Kamar, P. Gatault, F. Petitprez, L. Couzi, N. Jourde-Chiche, V. Châtelet, Raphaël Gaisne, D. Bertrand, J. Bamoulid, Magali Louis, R. Sberro Soussan, D. Navarro, P. Westeel, L. Frimat, C. Colosio, A. Thierry, J. Rivalan, L. Albano, N. Arzouk, E. Cornec-Le Gall, G. Claisse, M. Elias, K. El Karoui, S. Chauvet, J. Coindre, J. Rerolle, L. Tricot, J. Sayegh, C. Garrouste, C. Charasse, Yahsou Delmas, Z. Massy, M. Hourmant, A. Servais, C. Loirat, F. Fakhouri, C. Pouteil‐Noble, M. Peraldi, C. Legendre, E. Rondeau, M. Le Quintrec, V. Frémeaux-Bacchi   +44 more
semanticscholar   +2 more sources

Atypical Hemolytic Uremic Syndrome: A Review of Complement Dysregulation, Genetic Susceptibility and Multiorgan Involvement. [PDF]

J Clin Med
Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA) characterized by complement dysregulation, leading to microvascular thrombosis and multi-organ injury.
Bogdan RG, Anderco P, Ichim C, Cimpean AM, Todor SB, Glaja-Iliescu M, Crainiceanu ZP, Popa ML.   +7 more
europepmc   +2 more sources

Atypical hemolytic uremic syndrome in high-risk neuroblastoma patient: case report

Онкогематология, 2021
Atypical hemolytic uremic syndrome is a rare disorder uncontrolled complement activation, which is classically manifested by anemia, thrombocytopenia and renal failure.
A. Yu. Smirnova, Yu. V. Dinikina, A. A. Tereshina, E. P. Evsutina, M. B. Belogurova   +4 more
doaj   +3 more sources

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   +7 more sources