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Atypical Hemolytic Uremic Syndrome

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https://www.readbyqxmd.com/read/28424685/analysis-of-linear-antibody-epitopes-on-factor-h-and-cfhr1-using-sera-of-patients-with-autoimmune-atypical-hemolytic-uremic-syndrome
#1
Eszter Trojnár, Mihály Józsi, Katalin Uray, Dorottya Csuka, Ágnes Szilágyi, Danko Milosevic, Vesna D Stojanović, Brankica Spasojević, Krisztina Rusai, Thomas Müller, Klaus Arbeiter, Kata Kelen, Attila J Szabó, György S Reusz, Satu Hyvärinen, T Sakari Jokiranta, Zoltán Prohászka
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). The functional consequence of FH-blockade is widely established; however, the molecular basis of autoantibody binding and the role of CFHR1 deficiency in disease pathogenesis are still unknown. We performed epitope mapping of FH to provide structural insight in the autoantibody recruitment on FH and potentially CFHR1...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28419995/hemolytic-uremic-syndrome-and-kidney-transplantation-a-case-series-and-review-of-the-literature
#2
Sabrina Milan Manani, Grazia Maria Virzì, Anna Giuliani, Anna Clementi, Alessandra Brocca, Daniela Dissegna, Francesca Martino, Emanuele Stefano Giovanni d'Amore, Claudio Ronco
BACKGROUND: Hemolytic uremic syndrome (HUS) can be triggered by Shiga toxin producing Escherichia coli (STEC) infection or it can be defined as atypical HUS (aHUS) if it is related to uncontrolled complement activation. aHUS is characterized by a high incidence of recurrence after kidney transplantation, and it can also occur de novo in transplant recipients. Eculizumab is used both to prevent and to treat aHUS following kidney transplantation. In this paper, we report our centre experience and we present 4 cases of HUS in patients who underwent kidney transplantation...
April 19, 2017: Nephron
https://www.readbyqxmd.com/read/28416508/hus-and-atypical-hus
#3
T Sakari Jokiranta
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy characterized by intravascular hemolysis, thrombocytopenia, and acute kidney failure. HUS is usually categorized as typical, caused by shiga-toxin producing E. coli (STEC) infection, atypical HUS (aHUS), usually caused by uncontrolled complement activation, or secondary HUS with a coexisting disease. In recent years, a general understanding of the pathogenetic mechanisms driving HUS has increased. Typical (i.e. STEC-HUS) follows a gastrointestinal infection with STEC, while aHUS is associated primarily with mutations or autoantibodies leading to dysregulated complement activation...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28405075/acquired-thrombotic-thrombocytopenic-purpura-and-atypical-hemolytic-uremic-syndrome-successfully-treated-with-eculizumab
#4
Appalanaidu Sasapu, Michele Cottler-Fox, Pooja Motwani
Acquired idiopathic thrombotic thrombocytopenic purpura is a life-threatening disease with a mortality of up to 90%, if not promptly recognized and treated. We report a 64-year-old woman with this condition who presented with left-sided weakness and seizure-like activity preceded by headache and easy bruising. She did not achieve optimal response to plasma exchange, corticosteroids, rituximab, and vincristine. We initiated treatment with eculizumab, following which she had durable remission that continued for 30 months after discontinuation of the drug...
April 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28391332/many-drugs-for-many-targets-novel-treatments-for-complement-mediated-glomerular-disease
#5
Joshua M Thurman
There is a large body of experimental and clinical evidence that complement activation contributes to glomerular injury in multiple different diseases. However, the underlying mechanisms that trigger complement activation vary from disease to disease. Immune complexes activate the classical pathway of complement in many types of glomerulonephritis, whereas the alternative pathway and mannose-binding lectin pathways are directly activated in some diseases. Eculizumab is an inhibitory antibody to C5 that has been approved for the treatment of atypical hemolytic uremic syndrome, and case reports suggest that it is also effective in other types of glomerulonephritis...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28387984/practical-issues-in-using-eculizumab-for-children-with-atypical-hemolytic-uremic-syndrome-in-the-acute-phase-a-review-of-4-patients
#6
Chikako Terano, Kenji Ishikura, Riku Hamada, Yasuhiro Yoshida, Wataru Kubota, Yusuke Okuda, Shunsuke Shinozuka, Ryoko Harada, Sunao Iyoda, Yoshihiro Fujimura, Yuko Hamasaki, Hiroshi Hataya, Masataka Honda
AIM: Recently eculizumab, a monoclonal antibody to C5, was found to improve the disease course of atypical hemolytic uremic syndrome (aHUS) and has been recommended as the first line treatment by an international consensus guideline. However, several practical issues in the use of eculizumab for the acute phase of aHUS have yet to be resolved. METHODS: Children who received eculizumab with diagnosis of aHUS between March 2010 and December 2015 at Tokyo Metropolitan Children's Medical Center were enrolled...
April 7, 2017: Nephrology
https://www.readbyqxmd.com/read/28381151/omental-arteriopathy-in-primary-atypical-hemolytic-uremic-syndrome
#7
Natalie Ellington, Matthias T F Wolf, Jennifer Kasten, Dinesh Rakheja
No abstract text is available yet for this article.
March 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28366510/controlling-the-anaphylatoxin-c5a-in-diseases-requires-a-specifically-targeted-inhibition
#8
Niels C Riedemann, Maria Habel, Jana Ziereisen, Marlen Hermann, Conny Schneider, Cyrill Wehling, Michael Kirschfink, Karim Kentouche, Renfeng Guo
The terminal complement split product C5a has been described as an important mediator in inflammatory diseases. C5a is generated upon cleavage of C5 and earlier research suggests that, besides the known C5 convertases formed upon activation of the complement pathways, various enzymes could activate C5 directly. We demonstrate that eculizumab effectively blocks C5 activation when mediated by C5-convertase formation, but fails to block C5a generation resulting from direct enzymatic cleavage by trypsin and thrombin...
March 30, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28356668/targeting-renin-angiotensin-system-in-malignant-hypertension-in-atypical-hemolytic-uremic-syndrome
#9
V Raghunathan, S K Sethi, M A Dragon-Durey, M Dhaliwal, R Raina, P Jha, S B Bansal, V Kher
Hypertension is common in hemolytic uremic syndrome (HUS) and often difficult to control. Local renin-angiotensin activation is believed to be an important part of thrombotic microangiopathy, leading to a vicious cycle of progressive renal injury and intractable hypertension. This has been demonstrated in vitro via enhanced tissue factor expression on glomerular endothelial cells which is enhanced by angiotensin II. We report two pediatric cases of atypical HUS with severe refractory malignant hypertension, in which we targeted the renin-angiotensin system by using intravenous (IV) enalaprilat, oral aliskiren, and oral enalapril with quick and dramatic response of blood pressure...
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28353273/clinical-characteristics-of-children-with-hemolytic-uremic-syndrome-in-hangzhou-china
#10
Shui-Ai Zhao, Bo-Tao Ning, Jian-Hua Mao
BACKGROUND: Hemolytic uremic syndrome (HUS) is a main cause of acute renal failure in children. This study aimed to analyze the clinical characteristics of HUS. METHODS: A retrospective analysis was performed in 46 children with sporadic HUS. RESULTS: Of the 46 HUS patients, 20 (43.5%) were diarrhea-related HUS, and 26 (56.5%) were atypical HUS. Anemia, edema, oliguria, hemoglobinuria and hypertension were the most common manifestations. Thrombocytopenia, hyponatremia, hypocalcemia, hyperkalemia, metabolic acidosis, increased fibrinogen and hypocomplementemia were found in most patients...
April 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28352025/eculizumab-experience-in-an-adult-patient-with-atypical-hemolytic-uremic-syndrome
#11
Funda Sari, Ayça Inci, Volkan Karakus, Banu Yilmaz, Metin Sarikaya, Refik Olmaz, Erdal Kurtoglu
Atypical hemolytic-uremic syndrome is a disease characterized by nonimmune hemolytic anemia, thrombocytopenia, and renal failure. In this study, we present a case of a patient with atypical hemolytic-uremic syndrome treated successfully with eculizumab. A 20-year-old female was admitted with clinical signs of atypical hemolytic syndrome. The laboratory findings were as follows: hemoglobin 9.2 g/dL, platelet count 18 × 103/μL, creatinine 4.69 mg/dL, schistocytes were in peripheral blood smear, lactate dehydrogenase 2080 U/L, and emergency plasmapheresis procedure with fresh frozen plasma were initiated...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28343354/unusual-severe-case-of-hemolytic-uremic-syndrome-due-to-shiga-toxin-2d-producing-e-coli-o80-h2
#12
Kioa L Wijnsma, Anne M Schijvens, John W A Rossen, A M D Mirjam Kooistra-Smid, Michiel F Schreuder, Nicole C A J van de Kar
BACKGROUND: Hemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in children, with the majority of cases caused by an infection with Shiga toxin-producing Escherichia coli (STEC). Whereas O157 is still the predominant STEC serotype, non-O157 serotypes are increasingly associated with STEC-HUS. However, little is known about this emerging and highly diverse group of non-O157 serotypes. With supportive therapy, STEC-HUS is often self-limiting, with occurrence of chronic sequelae in just a small proportion of patients...
March 25, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28341889/complement-regulation-and-kidney-diseases-recent-knowledge-of-the-double-edged-roles-of-complement-activation-in-nephrology
#13
REVIEW
Masashi Mizuno, Yasuhiro Suzuki, Yasuhiko Ito
The complement activation system plays important roles to maintain homeostasis in the host and to fight foreign invaders to protect the host. Therefore, the complement system is considered a core part of innate immunity which also cross-talks to acquired immunity. In the history of nephrology, the complement system is familiar to us, because complement protein or fragment deposition, including C3, C4, C1q, and/or C4d, is routinely estimated by immunohistochemistry to diagnose renal pathologies. The relationships between pathological mechanisms and complement activation have been investigated for renal diseases such as post-infectious glomerulonephritis, lupus nephritis, and primary membranoproliferative glomerulonephritis, which are usually accompanied by hypocomplementemia...
March 24, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28320387/case-report-atypical-hemolytic-uremic-syndrome-triggered-by-influenza-b
#14
Robin Kobbe, Raphael Schild, Martin Christner, Jun Oh, Sebastian Loos, Markus J Kemper
BACKGROUND: Influenza A infections have been described to cause secondary hemolytic uremic syndrome and to trigger atypical hemolytic uremic syndrome (aHUS) in individuals with an underlying genetic complement dysregulation. To date, influenza B has not been reported to trigger aHUS. CASE PRESENTATION: A 6-month-old boy presented with hemolytic uremic syndrome triggered by influenza B infection. Initially the child recovered spontaneously. When he relapsed Eculizumab treatment was initiated, resulting in complete and sustained remission...
March 20, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28287636/transplant-associated-thrombotic-microangiopathy-opening-pandora-s-box
#15
REVIEW
E Gavriilaki, I Sakellari, A Anagnostopoulos, R A Brodsky
Transplant-associated thrombotic microangiopathy (TA-TMA) is an early complication of hematopoietic cell transplantation (HCT). A high mortality rate is documented in patients who are refractory to calcineurin inhibitor cessation. Estimates of TA-TMA prevalence vary significantly and are higher in allogeneic compared with autologous HCT. Furthermore, our understanding of the pathophysiology that is strongly related to diagnosis and treatment options is limited. Recent evidence has linked TA-TMA with atypical hemolytic uremic syndrome, a disease of excessive activation of the alternative pathway of complement, opening the Pandora's box in treatment options...
March 13, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28275964/reversal-of-threatening-blindness-after-initiation-of-eculizumab-in-purtscher-like-retinopathy-secondary-to-atypical-hemolytic-uremic-syndrome
#16
J E Ramos de Carvalho, R O Schlingemann, M Oranje, F J Bemelman, M J van Schooneveld
Purtscher-like retinopathy, a rare manifestation of systemic thrombotic microangiopathy, is a potentially visually debilitating condition with no effective proven treatment. Distinct pathogenic pathways have been proposed as etiological factors. We revisit the etiology of Purtscher-like retinopathy based on the rapid response and profound visual improvement after initiation of systemic intravenous eculizumab, an inhibitor of the complement cascade, in a patient with Purtscher-like retinopathy secondary to familial atypical hemolytic uremic syndrome (aHUS) due to a mutation in complement factor H...
March 8, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28250608/complements-spurned-our-experience-with-atypical-hemolytic-uremic-syndrome
#17
Vidya S Nagar, Rudrarpan Chaterjee, Ankita Sood, Basavaraj Sajjan, Aniruddha Kaushik, Sameer V Vyahalkar
Atypical hemolytic uremic syndrome (aHUS) is a rare disorder resulting from a dysregulated activation of the alternative pathway of the complement system. It results in significant morbidity and mortality if not diagnosed and treated promptly. It lends itself to myriad renal and extrarenal manifestations, all potentially disabling. Eculizumab, a monoclonal antibody to complement C5 is now the widely accepted norm for treatment. However, in resource-limited settings, plasma exchange if instituted early may be as beneficial...
February 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28224376/a-case-of-atypical-hemolytic-uremic-syndrome-in-a-second-renal-transplant
#18
Nicholas A Zwang, Bing Ho, Yashpal S Kanwar, Brad Lewis, Matthew Cusick, John J Friedewald, Lorenzo Gallon
Atypical hemolytic uremic syndrome (aHUS) has gained increased visibility over several years as an important cause of renal failure. Unfortunately, diagnosis is often difficult because individual courses can be highly variable depending the causative genetic mutations. Here we present the case of a patient with a failed renal allograft and acute failure of a second allograft who was ultimately diagnosed with aHUS. Interestingly, he developed early de novo donor specific antibodies (DSA) after the second renal transplant in context of likely recurrent aHUS...
February 21, 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/28215731/complementopathies
#19
REVIEW
Andrea C Baines, Robert A Brodsky
The complement system is an essential part of the innate immune system that requires careful regulation to ensure responses are appropriately directed against harmful pathogens, while preventing collateral damage to normal host cells and tissues. While deficiency in some components of the complement pathway is associated with increased susceptibility to certain infections, it has also become clear that inappropriate activation of complement is an important contributor to human disease. A number of hematologic disorders are driven by complement, and these disorders may be termed "complementopathies"...
February 6, 2017: Blood Reviews
https://www.readbyqxmd.com/read/28210841/rare-genetic-variant-in-the-cfb-gene-presenting-as-atypical-hemolytic-uremic-syndrome-and-immune-complex-diffuse-membranoproliferative-glomerulonephritis-with-crescents-successfully-treated-with-eculizumab
#20
Khalid Alfakeeh, Mohammed Azar, Majid Alfadhel, Alsuayri Mansour Abdullah, Nourah Aloudah, Khaled O Alsaad
BACKGROUND: Complement factor B gene (CFB) is an important component of the alternate pathway of complement activation that provides an active subunit that associates with C3b to form the C3 convertase, which is an essential element in complement activation. Among the complement-associated disorders, mutations and pathogenic variants in the CFB gene are relatively rare phenomena. Moreover, mutated CFB affiliation with immune-complex diffuse membranoproliferative glomerulonephritis (IC-MPGN) and atypical hemolytic uremic syndrome (aHUS) are considered a highly rare occurrence...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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