Read by QxMD icon Read

Atypical hemolytic uremic syndrome

shared collection
8 papers 25 to 100 followers
By Faye Kehler Family Physician and GP Anesthetist since 1987 interested in all aspects of Medicine
Sarah E Sartain, Nancy A Turner, Joel L Moake
Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy with severe renal injury secondary to an overactive alternative complement pathway (AP). aHUS episodes are often initiated or recur during inflammation. We investigated gene expression of the surface complement regulatory proteins (CD55, CD59, CD46, and CD141 [thrombomodulin]) and AP components in human glomerular microvascular endothelial cells (GMVECs) and in HUVECs, a frequently used investigational model of endothelial cells. Surface complement regulatory proteins were also quantified by flow cytometry...
January 15, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
Gianluigi Ardissino, Francesca Tel, Ilaria Possenti, Sara Testa, Dario Consonni, Fabio Paglialonga, Stefania Salardi, Nicolò Borsa-Ghiringhelli, Patrizia Salice, Silvana Tedeschi, Pierangela Castorina, Rosaria Maria Colombo, Milena Arghittu, Laura Daprai, Alice Monzani, Rosangela Tozzoli, Maurizio Brigotti, Erminio Torresani
BACKGROUND: Hemolytic uremic syndrome associated with Shiga toxin-producing Escherichia coli (STEC-HUS) is a severe acute illness without specific treatment except supportive care; fluid management is concentrated on preventing fluid overload for patients, who are often oligoanuric. Hemoconcentration at onset is associated with more severe disease, but the benefits of volume expansion after hemolytic uremic syndrome (HUS) onset have not been explored. METHODS: All the children with STEC-HUS referred to our center between 2012 and 2014 received intravenous infusion targeted at inducing an early volume expansion (+10% of working weight) to restore circulating volume and reduce ischemic or hypoxic tissue damage...
January 2016: Pediatrics
Elizabeth C Schramm, Lubka T Roumenina, Tania Rybkine, Sophie Chauvet, Paula Vieira-Martins, Christophe Hue, Tara Maga, Elisabetta Valoti, Valerie Wilson, Sakari Jokiranta, Richard J H Smith, Marina Noris, Tim Goodship, John P Atkinson, Veronique Fremeaux-Bacchi
The pathogenesis of atypical hemolytic uremic syndrome (aHUS) is strongly linked to dysregulation of the alternative pathway of the complement system. Mutations in complement genes have been identified in about two-thirds of cases, with 5% to 15% being in C3. In this study, 23 aHUS-associated genetic changes in C3 were characterized relative to their interaction with the control proteins factor H (FH), membrane cofactor protein (MCP; CD46), and complement receptor 1 (CR1; CD35). In surface plasmon resonance experiments, 17 mutant recombinant proteins demonstrated a defect in binding to FH and/or MCP, whereas 2 demonstrated reduced binding to CR1...
April 9, 2015: Blood
Spero R Cataland, Haifeng M Wu
Published data demonstrating the efficacy of complement inhibition therapy in patients with atypical hemolytic uremic syndrome (aHUS) are remarkable in contrast to the historically poor long-term prognosis for aHUS patients treated with plasma-based therapy. Although both aHUS and acquired thrombotic thrombocytopenic purpura (TTP) remain clinical diagnoses, an increased understanding of both conditions has improved our ability to differentiate aHUS from acquired TTP. These same data have also demonstrated the importance of a more rapid identification and diagnosis of aHUS as the recovery of end-organ injury present appears to be related to the time to initiate therapy with eculizumab...
April 17, 2014: Blood
Fadi Fakhouri, Yahsou Delmas, François Provot, Christelle Barbet, Alexandre Karras, Raifah Makdassi, Cécile Courivaud, Khair Rifard, Aude Servais, Catherine Allard, Virginie Besson, Maud Cousin, Valérie Châtelet, Jean-Michel Goujon, Jean-Philippe Coindre, Guillaume Laurent, Chantal Loirat, Véronique Frémeaux-Bacchi
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a devastating form of renal thrombotic microangiopathy. Despite plasma exchange, the standard treatment of aHUS for decades, the renal prognosis for patients with aHUS has remained poor. We assessed the off-trial use of eculizumab in adult patients with aHUS affecting the native kidneys. STUDY DESIGN: A retrospective study was conducted. aHUS was defined as the presence of 3 or more of the following: acute kidney injury (serum creatinine >1...
January 2014: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Han-Mou Tsai
Since the last review in 2007 of thrombotic thrombocytopenic purpura (TTP) and microangiopathic hemolytic anemia in the Clinics, further understanding of the nature of TTP and atypical hemolytic uremic syndrome (aHUS) has led to increasing use of rituximab in the treatment of TTP and the approval in 2011 of eculizumab for the treatment of aHUS. With this new armamentarium, distinction of aHUS from TTP has become more critical than ever. This article updates the new knowledge, highlights the difference between aHUS and TTP, and presents a scheme for their diagnosis and management...
June 2013: Hematology/oncology Clinics of North America
Marie Frimat, Fanny Tabarin, Jordan D Dimitrov, Caroline Poitou, Lise Halbwachs-Mecarelli, Veronique Fremeaux-Bacchi, Lubka T Roumenina
Atypical hemolytic uremic syndrome (aHUS) is characterized by genetic and acquired abnormalities of the complement system leading to alternative pathway (AP) overactivation and by glomerular endothelial damage, thrombosis, and mechanical hemolysis. Mutations per se are not sufficient to induce aHUS, and nonspecific primary triggers are required for disease manifestation. We investigated whether hemolysis-derived heme contributes to aHUS pathogenesis. We confirmed that heme activates complement AP in normal human serum, releasing C3a, C5a, and sC5b9...
July 11, 2013: Blood
Elena Bresin, Erica Rurali, Jessica Caprioli, Pilar Sanchez-Corral, Veronique Fremeaux-Bacchi, Santiago Rodriguez de Cordoba, Sheila Pinto, Timothy H J Goodship, Marta Alberti, David Ribes, Elisabetta Valoti, Giuseppe Remuzzi, Marina Noris
Several abnormalities in complement genes reportedly contribute to atypical hemolytic uremic syndrome (aHUS), but incomplete penetrance suggests that additional factors are necessary for the disease to manifest. Here, we sought to describe genotype-phenotype correlations among patients with combined mutations, defined as mutations in more than one complement gene. We screened 795 patients with aHUS and identified single mutations in 41% and combined mutations in 3%. Only 8%-10% of patients with mutations in CFH, C3, or CFB had combined mutations, whereas approximately 25% of patients with mutations in MCP or CFI had combined mutations...
February 2013: Journal of the American Society of Nephrology: JASN
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"