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Atypical HUS

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https://www.readbyqxmd.com/read/28419995/hemolytic-uremic-syndrome-and-kidney-transplantation-a-case-series-and-review-of-the-literature
#1
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
#2
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/28387984/practical-issues-in-using-eculizumab-for-children-with-atypical-hemolytic-uremic-syndrome-in-the-acute-phase-a-review-of-4-patients
#3
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/28356668/targeting-renin-angiotensin-system-in-malignant-hypertension-in-atypical-hemolytic-uremic-syndrome
#4
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
#5
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/28347544/ocular-involvement-in-atypical-haemolytic-uraemic-syndrome
#6
A Sampedro López, B Domínguez Moro, J M Baltar Martin, C Garcia Monteavaro, J J Barbón García
CASE REPORT: The case is presented of a young man with an atypical haemolytic-uraemic syndrome (aHUS), complicated with bilateral serous retinal detachment, cotton wool spots, and a branch artery occlusion. Treatment with plasmapheresis, haemodialysis and systemic eculizumab led to the blood and urine parameters returning to normal, as well as resolution of the retinal anomalies. Genetic analysis show both mutations in complement factor H and C3. DISCUSSION: Haemolytic-uraemic syndrome (HUS) is a thrombotic microangiopathy characterised by microangiopathic haemolytic anaemia, thrombocytopenia, and acute renal failure...
March 24, 2017: Archivos de la Sociedad Española de Oftalmología
https://www.readbyqxmd.com/read/28343354/unusual-severe-case-of-hemolytic-uremic-syndrome-due-to-shiga-toxin-2d-producing-e-coli-o80-h2
#7
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/28295239/eculizumab-dosing-regimen-in-atypical-hus-possibilities-for-individualized-treatment
#8
Elena Volokhina, Kioa Wijnsma, Renate van der Molen, Nel Roeleveld, Thea van der Velden, Joop Goertz, Fred Sweep, Roger J Brüggemann, Jack Wetzels, Nicole van de Kar, Lambertus van den Heuvel
Recent studies indicate that eculizumab is often given in excess to aHUS patients. Individualization of treatment is thus highly requested, however, data on pharmacokinetics and pharmacodynamics of eculizumab remain limited. We analyzed 11 patients during induction (weekly), maintenance (2-weekly) and tapering (every 3-8 weeks) phases of treatment. The trough eculizumab levels increased with each additional dose during the induction phase (depending on body weight). During maintenance, high eculizumab concentrations of up to 772 µg/mL were observed...
March 15, 2017: Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/28178155/complement-mediated-thrombotic-microangiopathy-secondary-to-sepsis-induced-disseminated-intravascular-coagulation-successfully-treated-with-eculizumab-a-case-report
#9
Tomohiro Abe, Akira Sasaki, Taichiro Ueda, Yoshitaka Miyakawa, Hidenobu Ochiai
Secondary thrombotic microangiopathies (TMAs) are induced by several underlying conditions; most are resolved by treating background disease. Eculizumab is a human monoclonal antibody that blocks the final stage of the complement system and effectively treats atypical hemolytic uremic syndrome (aHUS). In this report, we present a patient with TMA secondary to sepsis- induced coagulopathy, who was successfully treated with eculizumab.A 44-year-old woman, who had no special medical history or familial history of TMAs, was admitted on suspicion of septic shock...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28177095/-atypical-hemolytic-uremic-syndrome-related-to-oxalyplatin-cancer-chemotherapy-responsive-to-eculizumab
#10
Fulvia Zanchelli, Elena Tampieri, Francesco Gozzetti, Mattia Monti, Davide Martelli, Romina Graziani, Eliana Zuffa, Daniele Vincenzi, Alessandro Gamboni, Andrea Buscaroli
We describe the case of a patient with adenocarcinoma of the colon treated with FOLFOX-4 (5-Fluorouracil, Folinic acid, Oxalyplatin), with subsequent appearance of atypical hemolytic uremic syndrome (aHUS). From 1999 to 2009, 13 cases of atypical HUS receiving chemotherapy with oxaliplatin have been described, as well as some sporadic cases. None of these cases has been treated with eculizumab. This is the first report of a patient with aHUS secondary to Oxalyplatin treated with Eculizumab. This treatment induced a complete remission of the syndrome and, later on, it has been discontinued with clinical and laboratory permanent remission...
January 2017: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/28176475/life-threatening-pregnancy-associated-atypical-haemolytic-uraemic-syndrome-and-its-response-to-eculizumab
#11
REVIEW
Ryan Gately, Aye San, Jagadeesh Kurtkoti, Alan Parnham
Pregnancy-associated atypical haemolytic uraemic syndrome (P-aHUS) is a rare, potentially lethal condition that can complicate pregnancy in up to 1 in 25 000 cases. Without prompt diagnosis and initiation of appropriate treatment, this condition can lead to disastrous consequences for both mother and child. Given the broad spectrum of conditions that can present similarly in the peripartum period, it is often difficult to establish the correct diagnosis in a timely manner. Recently, the terminal complement cascade inhibitor eculizumab has been used with considerable success in non-pregnancy HUS; however, its use in P-aHUS is limited to isolated case reports...
February 2017: Nephrology
https://www.readbyqxmd.com/read/28110418/occurrence-of-atypical-hus-associated-with-influenza-b
#12
Karen van Hoeve, Corinne Vandermeulen, Marc Van Ranst, Elena Levtchenko, Lambert van den Heuvel, Djalila Mekahli
Hemolytic uremic syndrome (HUS) is a disease characterized by thrombotic microangiopathy with a triad of non-immune hemolytic anemia, thrombocytopenia, and renal impairment. Approximately 10% of cases of HUS are classified as atypical (aHUS). While today many genetically forms of aHUS pathology are known, only about 50% of carriers precipitate the disease. The reason remains unclear, and triggering events like intercurrent infections have been postulated. In rare cases, influenza A is the known trigger of aHUS; however, no cases of influenza B have been reported...
January 21, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28058212/complement-related-kidney-diseases-recurrence-after-transplantation
#13
REVIEW
Maurizio Salvadori, Elisabetta Bertoni
The recurrence of renal disease after renal transplantation is becoming one of the main causes of graft loss after kidney transplantation. This principally concerns some of the original diseases as the atypical hemolytic uremic syndrome (HUS), the membranoproliferative glomerulonephritis (MPGN), in particular the MPGN now called C3 glomerulopathy. Both this groups of renal diseases are characterized by congenital (genetic) or acquired (auto-antibodies) modifications of the alternative pathway of complement...
December 24, 2016: World Journal of Transplantation
https://www.readbyqxmd.com/read/27976568/an-unexpected-diagnostic-course-of-systemic-lupus-erythematosus
#14
Aysel Taktak, Adem Köksoy, Özge Başaran, Saba Kiremitçi, Banu Acar, Nermin Uncu, Nilgün Çakar
Thrombotic microangiopathy (TM), especially thrombotic thrombocytopenic purpura (TTP) is described in systemic lupus erythematosus (SLE) as a severe hematological involvement. However hemolytic uremic syndrome (HUS) is seen less frequently in SLE, particularly as an initial presentation. Here we present a 15-year old boy presenting with gross hematuria, decreased urinary output and petechial lesions. He was diagnosed as atypical HUS according to the classical triad of TM, along with observation of hypocomplementemia and negative stool cultures...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27951532/brief-review-and-a-clinical-case-of-hemolytic-uremic-syndrome-associated-with-interferon-%C3%AE-treatment
#15
Sabrina Milan Manani, Grazia Maria Virzì, Fiorella Gastaldon, Marta Proglio, Alessandra Brocca, Claudio Ronco
The hemolytic uremic syndrome (HUS) is one of the thrombotic microangiopathies and it consists of the triad of nonimmune microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. The atypical form of HUS (aHUS) is related to causative mutations in complement genes. Some conditions act as a trigger for aHUS in individuals that have a genetic background predisposing to complement activation. Interferon β is a recombinant-protein therapy approved to treat multiple sclerosis (MS), and can be a causative agent in the occurrence of HUS through anti-angiogenic activity...
2017: Blood Purification
https://www.readbyqxmd.com/read/27930620/atypical-hemolytic-uremic-syndrome-ahus-essential-aspects-of-an-accurate-diagnosis
#16
REVIEW
Jeffrey Laurence, Hermann Haller, Pier Mannuccio Mannucci, Masaomi Nangaku, Manuel Praga, Santiago Rodriguez de Cordoba
Atypical hemolytic uremic syndrome (aHUS), a thrombotic microangiopathy (TMA), is a rare, life-threatening, systemic disease. When unrecognized or inappropriately treated, aHUS has a high degree of morbidity and mortality. aHUS results from chronic, uncontrolled activity of the alternative complement pathway, which activates platelets and damages the endothelium. Two-thirds of aHUS cases are associated with an identifiable complement-activating condition. aHUS is clinically very similar to the other major TMAs: Shiga toxin-producing Escherichia coli (STEC)-HUS, thrombotic thrombocytopenic purpura (TTP), and disseminated intravascular coagulation (DIC)...
November 2016: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/27904864/nephrotic-range-proteinuria-and-peripheral-edema-in-a-child-not-only-idiopathic-nephrotic-syndrome
#17
Valentina Dolcemascolo, Marina Vivarelli, Manuela Colucci, Francesca Diomedi-Camassei, Rossella Piras, Marta Alberti, Francesco Emma
Hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy (TMA) mainly occurring in renal and cerebral microvessels. Although the most common cause of HUS in children is Shiga toxin-producing Escherichia coli, atypical forms in which Shiga toxin is not the trigger may occur. Research over the last few years has shown that complement dysregulation secondary to mutations of genes coding for proteins involved in the regulation of the alternative pathway of complement account for most forms of atypical HUS (aHUS)...
September 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27787280/acute-kidney-injury-in-a-patient-with-hemolytic-anemia-and-thrombocytopenia
#18
Michael Breunig, Miguel Lalama, Gabrielle Rivard, Deanne Kashiwagi, Lynn Cornell
Atypical hemolytic uremic syndrome (HUS) is clinically difficult to distinguish from HUS and thrombotic thrombocytopenic purpura. Atypical HUS results from dysregulation of complement activation causing thrombotic microangiopathy affecting multiple organ systems. Atypical HUS is associated with high morbidity and mortality, making early recognition and appropriate therapy necessary to improve patient outcomes.
November 2016: JAAPA: Official Journal of the American Academy of Physician Assistants
https://www.readbyqxmd.com/read/27781079/atypical-hemolytic-uremic-syndrome-secondary-to-lupus-nephritis-responsive-to-eculizumab
#19
Alexander G Raufi, Shruti Scott, Omar Darwish, Kevin Harley, Kanwarpal Kahlon, Sheetal Desai, Yuxin Lu, Minh-Ha Tran
Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) - a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage...
September 28, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27768015/hemolytic-uremic-syndrome-in-children
#20
Valentina Talarico, Monica Aloe, Alice Monzani, Roberto Miniero, Gianni Bona
Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by thrombocytopenia, non-immune microangiopathic hemolytic anemia and acute renal failure. HUS is typically classified into two primary types: 1) HUS due to infections, often associated with diarrhea (D+HUS, Shiga toxin-producing Escherichia Coli-HUS), with the rare exception of HUS due to a severe disseminated infection caused by Streptococcus; 2) HUS related to complement, such HUS is also known as "atypical HUS" and is not diarrhea associated (D-HUS, aHUS); but recent studies have shown other forms of HUS, that can occur in the course of systemic diseases or physiopathological conditions such as pregnancy, after transplantation or after drug assumption...
December 2016: Minerva Pediatrica
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