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HUS, ESRD, Renal transplant

Karolis Azukaitis, Eva Simkova, Mohammad Abdul Majid, Matthias Galiano, Kerstin Benz, Kerstin Amann, Clemens Bockmeyer, Radha Gajjar, Kevin E Meyers, Hae Il Cheong, Bärbel Lange-Sperandio, Therese Jungraithmayr, Véronique Frémeaux-Bacchi, Carsten Bergmann, Csaba Bereczki, Monika Miklaszewska, Dorottya Csuka, Zoltán Prohászka, Patrick Gipson, Matthew G Sampson, Mathieu Lemaire, Franz Schaefer
The recent discovery of mutations in the gene encoding diacylglycerol kinase ε (DGKE) identified a novel pathophysiologic mechanism leading to HUS and/or MPGN. We report ten new patients from eight unrelated kindreds with DGKE nephropathy. We combined these cases with all previously published cases to characterize the phenotypic spectrum and outcomes of this new disease entity. Most patients presented with HUS accompanied by proteinuria, whereas a subset of patients exhibited clinical and histologic patterns of MPGN without TMA...
October 2017: Journal of the American Society of Nephrology: JASN
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...
2017: Nephron
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
Zhimin Chen, Abdul Rashid Qureshi, Jonaz Ripsweden, Lars Wennberg, Olof Heimburger, Bengt Lindholm, Peter Barany, Mathias Haarhaus, Torkel B Brismar, Peter Stenvinkel
OBJECTIVE: Chronic kidney disease-mineral bone disorder (CKD-MBD) is a major complication of end-stage renal disease (ESRD). Reduced bone mineral density (BMD) is associated with vascular calcification. Here we investigated associations between vertebral bone density (VBD) and coronary artery calcification (CAC), quantified by cardiac computed tomography (CT), and BMD quantified by dual-energy X-ray absorptiometry (DXA), and their relations with mortality. METHODS: In 231 ESRD patients (median age 56years, 63% males) comprising incident dialysis patients, prevalent peritoneal dialysis patients and recipients of living donor kidney transplant, VBD (Hounsfield units, HUs) and CAC scores (Agatston units, AUs) were quantified by cardiac CT, and, in 143 of the patients, BMD was measured by DXA of total body...
November 2016: Bone
Umut Kasapoğlu, Çaglar Ruhi, Murat Tuğcu, Başak Boynueğri, İzzet Titiz, Veysel Sabri Hançer, Süheyla Apaydın
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a very rare disease, which presents with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Progression to end-stage renal disease (ESRD) from acute kidney injury is observed in 60% of aHUS cases. The prognosis of aHUS patients who undergo kidney transplantation (Ktx) is generally poor, but these patients should be treated prophylactically with eculizumab to prevent recurrence after transplantation. CASE REPORT: An 18-year-old man was referred to our center with a history of rapid progression to ESRD with unknown etiology...
December 1, 2015: Annals of Transplantation: Quarterly of the Polish Transplantation Society
Alfonso H Santos, Michael J Casey, Xuerong Wen, Ivan Zendejas, Shehzad Rehman, Karl L Womer, Kenneth A Andreoni
BACKGROUND: Survival data are lacking for kidney transplant recipients with rare native end-stage renal disease (ESRD) etiologies. There is currently no large registry study comparing dialysis versus kidney transplantation survival outcomes of waitlisted adults with hemolytic uremic syndrome (HUS). MATERIALS AND METHODS: We retrospectively studied adult-HUS end-stage renal disease patients (n = 559) placed on the US kidney transplant waitlist in 1996 to 2011. We analyzed 5-year transplantation and patient survival probabilities and risk factors using Kaplan-Meier and Cox hazards models, respectively...
December 2015: Transplantation
Johannes Hofer, Thomas Giner, Gerard Cortina, Therese Jungraithmayr, Jurate Masalskiene, Diana Dobiliene, Renata Mitkiene, Birute Pundziene, Sarunas Rudaitis
CFH-Ab-associated aHUS requires different diagnostic and therapeutic approaches and then the genetically defined aHUS forms. The risk of post-transplant recurrence with graft dysfunction in CFH-Ab aHUS is not well documented. It is suggested that recurrence can be expected if a significant CFH-Ab load persists at the time of transplantation. A pretransplant procedure to reduce CFH-Ab titer seems reasonable, but accurate recommendations are lacking. Whether further prophylactic interventions after transplantation are necessary has to be decided on an individual basis...
August 2015: Pediatric Transplantation
Keiko Kawaguchi, Kunio Kawanishi, Masayo Sato, Mitsuyo Itabashi, Akiko Fujii, Yukiko Kanetsuna, Shouhei Huchinoue, Ryuji Ohashi, Junki Koike, Kazuho Honda, Yoji Nagashima, Kosaku Nitta
Atypical hemolytic uremic syndrome (aHUS) in allograft kidney transplantation is caused by various factors including rejection, infection, and immunosuppressive drugs. We present a case of a 32 year old woman with aHUS four years after an ABO-incompatible kidney transplantation from a living relative. The primary cause of end-stage renal disease was unknown; however, IgA nephropathy (IgAN) was suspected from her clinical course. She underwent pre-emptive kidney transplantation from her 60 year old mother. The allograft preserved good renal function [serum creatinine (sCr) level 110-130 μmol/L] until a sudden attack of abdominal pain four years after transplant, with acute renal failure (sCr level, 385...
July 2015: Nephrology
Claire Rigothier, Yahsou Delmas, Lubka T Roumenina, Cécile Contin-Bordes, Sébastien Lepreux, Frank Bridoux, Jean Michel Goujon, Thomas Bachelet, Guy Touchard, Véronique Frémeaux-Bacchi, Christian Combe
Abnormal regulation of the alternative pathway of the complement system is a well-described trigger of microangiopathy leading to atypical hemolytic uremic syndrome (aHUS). However, the involvement of complement dysregulation in distal angiopathy has not been reported in adults. We describe the clinical course of a patient with severe distal angiopathy (amputation of all fingers and toes) followed 3 years later by aHUS with end-stage renal disease. This course was attributed to a circulating monoclonal immunoglobulin A λ light chain (IgAλ) with unusual properties: it bound complement factor H (CFH) and impaired CFH-glycosaminoglycan interaction and cell-surface protection...
August 2015: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
Donal J Sexton, Scott Reule, Craig A Solid, Shu-Cheng Chen, Allan J Collins, Robert N Foley
Management of hemolytic uremic syndrome (HUS) has evolved rapidly, and optimal treatment strategies are controversial. However, it is unknown whether the burden of end-stage renal disease (ESRD) from HUS has changed, and outcomes on dialysis in the United States are not well described. We retrospectively examined data for patients initiating maintenance renal replacement therapy (RRT) (n = 1,557,117), 1995-2010, to define standardized incidence ratios (SIRs) and outcomes of ESRD from HUS) (n = 2241)...
October 2015: Hemodialysis International
Hassan Salameh, Mohannad Abu Omar, Ahmad Alhariri, Sood Kisra, Abdulraheem Qasem, Aref Bin Abdulhak
Hemolytic uremic syndrome is the triad of nonimmune microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. When not associated with enteric infection, it is classified as atypical hemolytic uremic syndrome (aHUS) and carries a worse outcome with high mortality rate and up to 50% of the survivors will end up with end-stage renal disease. Renal transplant was restricted to a very small percentage of patients due to high recurrence rate posttransplant that approaches 90%. Our case describes a posttransplant adult patient with familial aHUS whom was successfully treated with eculizumab...
July 2016: American Journal of Therapeutics
Miquel Blasco Pelicano, Santiago Rodríguez de Córdoba, Josep M Campistol Plana
The hemolytic uremic syndrome (HUS) is a clinical entity characterized by thrombocytopenia, non-immune hemolytic anemia and renal impairment. Kidney pathology shows thrombotic microangiopathy (TMA) with endothelial cell injury leading to thrombotic occlusion of arterioles and capillaries. Traditionally, HUS was classified in 2 forms: Typical HUS, most frequently occurring in children and caused by Shiga-toxin-producing bacteria, and atypical HUS (aHUS). aHUS is associated with mutations in complement genes in 50-60% of patients and has worse prognosis, with the majority of patients developing end stage renal disease...
November 20, 2015: Medicina Clínica
Xiao-Juan Yu, Feng Yu, Di Song, Su-Xia Wang, Yan Song, Gang Liu, Ming-Hui Zhao
OBJECTIVE: The current study aimed to investigate the spectrum of etiologies and associated disorders of renal biopsy-proven thrombotic microangiopathy (TMA) patients. METHODS: The clinical, laboratory, and renal histopathological data of patients with renal TMA from 2000 to 2012 in our institute were collected and reviewed. RESULTS: One hundred and nine TMA patients were enrolled in this study. The mean age was 34.0 ± 11.1 years. Seventy patients (64...
2014: TheScientificWorldJournal
Priyanka Khandelwal, Aditi Sinha, Pankaj Hari, Virinder K Bansal, Amit K Dinda, Arvind Bagga
Atypical HUS associated with anti-CFH autoantibodies is an uncommon illness associated with high risk of progression to end-stage renal disease. Disease relapses after transplantation, observed in one-third cases, often lead to graft loss. We report four patients with anti-CFH antibody-associated HUS who underwent renal transplantation 16-62 months from initial presentation. Two patients each received organs from deceased and living-related donors. Anti-CFH antibody titers were monitored during the illness and following transplantation...
August 2014: Pediatric Transplantation
Ali Nayer, Arif Asif
Atypical hemolytic-uremic syndrome (HUS) is a rare life-threatening disorder characterized by microangiopathic hemolytic anemia, thrombocytopenia, and ischemic injury to organs, especially the kidneys. Microvascular injury and thrombosis are the dominant histologic findings. Complement activation through the alternative pathway plays a critical role in the pathogenesis of atypical HUS. Genetic abnormalities involving complement regulatory proteins and complement components form the molecular basis for complement activation...
January 2016: American Journal of Therapeutics
Véronique Frémeaux-Bacchi
Hemolytic Uremic Syndrome (HUS) is characterized by the triad of hemolytic anemia, thrombocytopenia and acute renal failure. The most frequent form in children is caused by Shiga-toxin producing Escherichia coli. In absence of Shiga-toxin infection, the HUS is called atypical (aHUS). Some HUS are secondary to Streptococcus pneumonia or human immunodeficiency virus infection, cancer, anti-cancer drugs, or cyclosporine. During the last decade, aHUS has been demonstrated to be a disorder of complement alternative pathway regulation...
2013: Biologie Aujourd'hui
Bartlomiej Posnik, Dorota Sikorska, Krzysztof Hoppe, Krzysztof Schwermer, Krzysztof Pawlaczyk, Andrzej Oko
Atypical hemolytic-uremic syndrome (aHUS), unlike typical HUS, is not due to bacteria but rather to an idiopathic or genetic cause that promotes dysregulation of the alternative complement pathway. It leads to hemolytic anemia, thrombocytopenia, and renal impairment. Although aHUS secondary to a genetic mutation is relatively rare, when occurring due to a mutation in Factor H (CFH), it usually presents with younger onset and has a more severe course, which in the majority ends with end-stage renal failure. Paradoxically to most available data, our case features acute aHUS due to a CFH mutation with late onset (38-year-old) and rapid progression to end-stage renal disease...
2013: Case Reports in Nephrology
Farzaneh Sharifipour, Abbasali Zeraati, Seyed Seifollah Beladi Mousavi, Fatemeh Hayati, Mohsen Tavazoe, Marzieh Beladi Mousavi
BACKGROUND: Two different case reports, which have been published previously, suggested that bilateral nephrectomy can improve sever and refractory hemolytic uremic syndrome (HUS) in adults without a history of transplantation. At this study, kidney transplant nephrectomy in a patient with sever post transplant HUS was investigated. CASE: Patient was a 55 years old man with a single small size kidney and end-stage renal disease (ESRD). He had received a kidney from an unrelated donor three months before admission...
January 2013: Journal of Nephropathology
Kathleen Webster, Eugene Schnitzler
The thrombotic microangiopathies include both hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Although debate exists as to whether these are separate entities or a spectrum of disease, both result in the clinical picture of thrombocytopenia, hemolytic anemia, and varying degrees of renal and neurologic involvement. Etiology of HUS includes diarrheal infection due to Shiga toxin-producing bacteria, complement deficiency, pneumococcal infection, and cobalamin deficiency. In disease ascribed to TTP, the main etiologic factor is deficiency of an enzyme known as a disintegrin-like and metalloprotease with thrombospondin type 1 repeats, number 13 (ADAMTS-13)...
2014: Handbook of Clinical Neurology
Jeffrey Saland
Patients with end-stage renal disease (ESRD) due to atypical HUS (aHUS) now have several potential options that can enable successful kidney transplantation. This editorial addresses these options by considering key factors that are important when making an individual treatment decision.
March 2014: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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