8,820 results match your criteria Hemolytic Uremic Syndrome

Genetic atypical hemolytic uremic syndrome in children: a 20-year experience from a tertiary center.

J Bras Nefrol 2021 May 12. Epub 2021 May 12.

Centro Hospitalar Universitário do Porto, Centro Materno-Infantil do Norte, Unidade de Nefrologia Pediátrica, Porto, Portugal.

Introduction: Atypical hemolytic uremic syndrome (aHUS) is a rare disorder characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, which primarily affects preschool-aged children. This study's aim was to describe the clinical profile, management, and long-term outcome of the genetic aHUS patients admitted to a tertiary care pediatric nephrology center during 20 years.

Methods: We performed a retrospective analysis of the clinical records of all aHUS patients younger than 18 years with identified genetic mutations. Read More

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Atypical Hemolytic-Uremic Syndrome Following Obstetric Hemorrhage in the Setting of Fetal Demise and Placenta Accreta: A Case Report.

A A Pract 2021 May 14;15(5):e01476. Epub 2021 May 14.

From the Department of Anesthesiology, Perioperative and Pain Medicine, Icahn School of Medicine at Mount Sinai, New York, New York.

Multiple disease processes can contribute to coagulopathy in the setting of intrauterine fetal demise. A 34-year-old woman with multiple prior uterine surgeries presented for dilation and evacuation of a fetal demise at 17 weeks. Her case was complicated by significant hemorrhage and coagulopathy requiring massive transfusion and hysterectomy. Read More

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Autoantibodies Against the Complement Regulator Factor H in the Serum of Patients With Neuromyelitis Optica Spectrum Disorder.

Front Immunol 2021 27;12:660382. Epub 2021 Apr 27.

Department of Immunology, ELTE Eötvös Loránd University, Budapest, Hungary.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disease of the central nervous system (CNS), characterized by pathogenic, complement-activating autoantibodies against the main water channel in the CNS, aquaporin 4 (AQP4). NMOSD is frequently associated with additional autoantibodies and antibody-mediated diseases. Because the alternative pathway amplifies complement activation, our aim was to evaluate the presence of autoantibodies against the alternative pathway C3 convertase, its components C3b and factor B, and the complement regulator factor H (FH) in NMOSD. Read More

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Loss of diacylglycerol kinase ε causes thrombotic microangiopathy by impairing endothelial VEGFA signaling.

JCI Insight 2021 May 10;6(9). Epub 2021 May 10.

Department of Internal Medicine, University of Iowa, Iowa City, Iowa, USA.

Loss of function of the lipid kinase diacylglycerol kinase ε (DGKε), encoded by the gene DGKE, causes a form of atypical hemolytic uremic syndrome that is not related to abnormalities of the alternative pathway of the complement, by mechanisms that are not understood. By generating a potentially novel endothelial specific Dgke-knockout mouse, we demonstrate that loss of Dgke in the endothelium results in impaired signaling downstream of VEGFR2 due to cellular shortage of phosphatidylinositol 4,5-biphosphate. Mechanistically, we found that, in the absence of DGKε in the endothelium, Akt fails to be activated upon VEGFR2 stimulation, resulting in defective induction of the enzyme cyclooxygenase 2 and production of prostaglandin E2 (PGE2). Read More

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It takes two to thrombosis: Hemolysis and complement.

Blood Rev 2021 Apr 30:100834. Epub 2021 Apr 30.

Department of Immunopathology, Sanquin Research and Landsteiner Laboratory, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands; Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Switzerland; Department for BioMedical Research, University of Bern, Switzerland. Electronic address:

Thromboembolic events represent the most common complication of hemolytic anemias characterized by complement-mediated hemolysis such as paroxysmal nocturnal hemoglobinuria and autoimmune hemolytic anemia. Similarly, atypical hemolytic uremic syndrome is characterized by hemolysis and thrombotic abnormalities. The main player in the development of thrombosis in hemolytic diseases is suggested to be the complement system. Read More

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Complement-Mediated Thrombotic Microangiopathy Associated with Lupus Nephritis Treated with Eculizumab: A Case Report.

Case Rep Nephrol Dial 2021 Jan-Apr;11(1):95-102. Epub 2021 Mar 31.

Division of Nephrology, Hypertension and Kidney Transplantation, Department of Medicine, University of California, Irvine, California, USA.

Thrombotic microangiopathies (TMAs) involve multiple organ systems due to the presence of microangiopathic hemolysis. One such condition, atypical hemolytic uremic syndrome (aHUS), is a complement-mediated process that is part of a spectrum of disorders that have underlying complement dysfunction of the alternative pathway due to overactivity or decreased self-nonself discrimination by innate immunity. Complement-amplifying conditions such as pregnancy may unmask a diagnosis of aHUS. Read More

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Atypical Evolution of Secondary Hemolytic Uremic Syndrome Defined as Paraneoplastic Syndrome under Eculizumab and Palbociclib Therapies.

Case Rep Oncol 2021 Jan-Apr;14(1):676-680. Epub 2021 Apr 12.

Nephrology, Hemodialysis, Apheresis and Kidney Transplantation Department, Grenoble Alpes University Hospital, Grenoble, France.

Thrombotic microangiopathy (TMA) is most of the time caused by thrombotic thrombocytopenic purpura or hemolytic uremic syndrome. A 60-year-old female was diagnosed in 2014 with mammary breast adenocarcinoma treated by several-line therapy: mastectomy, docetaxel, cyclophosphamide, radiotherapy, doxorubicine, and capecitabine. By mid-November, the patient was admitted to the hospital with regenerative, mechanical, and hemolytic anemia, schistocytes at 3%, and thrombopenia (99 G/L), associated with high blood transfusion requirement. Read More

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Primary Refractory Catastrophic Antiphospholipid Syndrome Masquerading as Buerger's Disease.

Cureus 2021 Apr 7;13(4):e14350. Epub 2021 Apr 7.

Division of Hematology and Oncology, University of California - Irvine, Irvine, USA.

Catastrophic antiphospholipid syndrome (CAPS) is a lethal disease with up to 30% mortality rate. It can occur as a primary disease or secondary to an underlying autoimmune disease. Current treatment focuses on disease control with anticoagulation and steroids. Read More

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Kidney transplant in patients with atypical hemolytic uremic syndrome in the anti-C5 era: single-center experience with tailored Eculizumab.

J Nephrol 2021 May 6. Epub 2021 May 6.

Kidney Transplant Unit, Center for HUS Prevention, Control and Management, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Rationale And Objective: Patients with atypical hemolytic uremic syndrome (aHUS) have long been considered ineligible for kidney transplantation (KTx) in several centers due to the high risk of disease recurrence, graft loss and life-threatening complications. The availability of Eculizumab (ECU) has now overcome this problem. However, the best approach towards timing, maintenance schedule, the possibility of discontinuation and patient monitoring has not yet been clearly established. Read More

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Thrombotic microangiopathy in aHUS and beyond: clinical clues from complement genetics.

Nat Rev Nephrol 2021 May 5. Epub 2021 May 5.

Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service d'Immunologie, Paris, France.

Studies of complement genetics have changed the landscape of thrombotic microangiopathies (TMAs), particularly atypical haemolytic uraemic syndrome (aHUS). Knowledge of complement genetics paved the way for the design of the first specific treatment for aHUS, eculizumab, and is increasingly being used to aid decisions regarding discontinuation of anti-complement treatment in this setting. Complement genetic studies have also been used to investigate the pathogenic mechanisms that underlie other forms of HUS and provided evidence that contributed to the reclassification of pregnancy- and postpartum-associated HUS within the spectrum of complement-mediated aHUS. Read More

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Whole-genome characterization of hemolytic uremic syndrome-causing Shiga toxin-producing in Sweden.

Virulence 2021 Dec;12(1):1296-1305

Division of Clinical Microbiology, Department of Laboratory Medicine, Karolinska Institutet, Huddinge, Sweden.

Shiga toxin-producing , a foodborne bacterial pathogen, has been linked to a broad spectrum of clinical outcomes ranging from asymptomatic carriage to fatal hemolytic uremic syndrome (HUS). Here, we collected clinical data and STEC strains from HUS patients from 1994 through 2018, whole-genome sequencing was performed to molecularly characterize HUS-associated STEC strains, statistical analysis was conducted to identify bacterial genetic factors associated with severe outcomes in HUS patients. O157:H7 was the most predominant serotype (57%) among 54 HUS-associated STEC strains, followed by O121:H19 (19%) and O26:H11 (7%). Read More

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December 2021

Tissue-resident macrophages mediate neutrophil recruitment and kidney injury in shiga toxin-induced hemolytic uremic syndrome.

Kidney Int 2021 Apr 28. Epub 2021 Apr 28.

Department of Immunodynamics, Institute of Experimental Immunology and Imaging, University Hospital Essen, Essen, Germany. Electronic address:

Enterohaemorrhagic E. coli cause major epidemics worldwide with significant organ damage and very high percentages of death. Due to the ability of enterohaemorrhagic E. Read More

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Shiga Toxin-Associated Hemolytic Uremic Syndrome: Specificities of Adult Patients and Implications for Critical Care Management.

Toxins (Basel) 2021 04 26;13(5). Epub 2021 Apr 26.

Centre de Référence des Microangiopathies Thrombotiques (CNR-MAT), Assistance Publique-Hôpitaux de Paris, Hôpital Saint-Antoine, 75012 Paris, France.

Shiga toxin-producing -associated hemolytic uremic syndrome (STEC-HUS) is a form of thrombotic microangiopathy secondary to an infection by an enterohemorrhagic . Historically considered a pediatric disease, its presentation has been described as typical, with bloody diarrhea at the forefront. However, in adults, the clinical presentation is more diverse and makes the early diagnosis hazardous. Read More

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Compound Haplotype Variants in and Genes Determine Clinical Outcome of Atypical Hemolytic Uremic Syndrome (aHUS)-A Series of Cases from a Single Family.

J Pers Med 2021 Apr 15;11(4). Epub 2021 Apr 15.

Warsaw Genomics INC, 01-682 Warsaw, Poland.

Atypical hemolytic uremic syndrome (aHUS) is a rare disease triggered by dysregulation of the alternative complement pathway, consisting of a characteristic triad of nonimmune hemolytic anemia, thrombocytopenia, and renal failure. The risk of aHUS onset, recurrence, and allograft loss depends on the genetic background of a patient. We show a series of cases from a single family whose five members were affected by aHUS and presented distinct clinical outcomes. Read More

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MALDI-TOF MS Based Typing for Rapid Screening of Multiple Antibiotic Resistance and Virulent Non-O157 Shiga Toxin-Producing Isolated from the Slaughterhouse Settings and Beef Carcasses.

Foods 2021 Apr 10;10(4). Epub 2021 Apr 10.

Public Health Department, College of Public Health and Health Informatics, Ha'il University, Ha'il 2440, Saudi Arabia.

Background: The emergence of multiple antibiotic resistance (MAR) ( and virulent non-O157 Shiga toxin-producing (STEC) poses a growing concern to the meat industry. Non-O157 STEC strains including O26, O45, O103, O111, O121, and O145 have been implicated in the occurrence of bloody diarrhea and hemolytic uremic syndrome in humans. This research assessed prevalence, matrix-assisted laser desorption/ionization-time of flight mass-spectrometry (MALDI-TOF MS) protein mass-spectra profiles, multidrug-resistance traits, polymerase chain reaction detection of virulence, and antibiotic-resistance genes of isolated from beef carcasses and slaughterhouse environments. Read More

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Functional and Genetic Landscape of Complement Dysregulation Along the Spectrum of Thrombotic Microangiopathy and its Potential Implications on Clinical Outcomes.

Kidney Int Rep 2021 Apr 3;6(4):1099-1109. Epub 2021 Feb 3.

Department of Nephrology and Clinical Immunology.

Introduction: The syndromes of thrombotic microangiopathy (TMA) are diverse and represent severe endothelial damage caused by various mechanisms. The complement system plays a major role in a subset of patients with TMA, and its recognition is of clinical importance because it guides choice and duration of treatment.

Methods: We studied a well-defined cohort of patients with TMA and hypothesized that assessment of serum-induced   C5b9 formation on the endothelium and screening for rare variants in complement genes can better categorize TMA. Read More

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Fighting by Blocking Its Disease-Causing Toxin.

J Med Chem 2021 May 28;64(9):6059-6069. Epub 2021 Apr 28.

Department of Chemical Biology & Drug Discovery, Utrecht Institute for Pharmaceutical Sciences, Utrecht University, Universiteitsweg 99, 3584 CG Utrecht, The Netherlands.

Shiga toxin is an AB toxin produced by species, while related toxins are produced by Shiga toxin-producing (STEC). Infection by Shigella can lead to bloody diarrhea followed by the often fatal hemolytic uremic syndrome (HUS). In the present paper, we aimed for a simple and effective toxin inhibitor by comparing three classes of carbohydrate-based inhibitors: glycodendrimers, glycopolymers, and oligosaccharides. Read More

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Whole-Genome Sequencing of Shiga Toxin-Producing Escherichia coli OX18 from a Fatal Hemolytic Uremic Syndrome Case.

Emerg Infect Dis 2021 May;27(5):1509-1512

We report a fatal case of hemolytic uremic syndrome with urinary tract infection in Japan caused by Shiga toxin-producing Escherichia coli. We genotypically identified the isolate as OX18:H2. Whole-genome sequencing revealed 3 potentially pathogenic lineages (OX18:H2, H19, and H34) that have been continuously isolated in Japan. Read More

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Thrombotic Microangiopathy After Kidney Transplantation: An Underdiagnosed and Potentially Reversible Entity.

Front Med (Lausanne) 2021 8;8:642864. Epub 2021 Apr 8.

Nephrology Department, University Hospital Dr. Peset, Valencia, Spain.

Thrombotic microangiopathy is a rare but serious complication that affects kidney transplant recipients. It appears in 0.8-14% of transplanted patients and negatively affects graft and patient survival. Read More

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Bevacizumab-associated thrombotic microangiopathy treated with eculizumab: A case series and systematic review of the literature.

Clin Nephrol 2021 Apr 26. Epub 2021 Apr 26.

Background: Bevacizumab is a recombinant monoclonal antibody against the vascular endothelial growth factor A (VEGF-A) ligand that is used in the management of various solid malignancies. The adverse effect profiles of angiogenesis inhibitors, such as bevacizumab, have become increasingly well characterized and include renal manifestations such as hypertension, proteinuria, and thrombotic microangiopathy. Eculizumab inhibits terminal-complement activation and is used to treat atypical hemolytic uremic syndrome. Read More

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Cerebral Microangiopathy in Two Dogs with Cutaneous and Renal Glomerular Vasculopathy.

J Comp Pathol 2021 Apr 8;184:95-100. Epub 2021 Apr 8.

Anderson Moores Veterinary Specialists, Hursley, Winchester, UK.

Cutaneous and renal glomerular vasculopathy (CRGV) is an emerging disease in the UK, but its aetiology remains unclear. It is considered a thrombotic microangiopathy (TMA) in which the kidney and skin are the most commonly affected organs. We now document two cases of CRGV with brain lesions, which may have accounted for neurological signs displayed by these animals. Read More

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Influenza-associated hemolytic uremic syndrome: The pathogenic role of the virus.

Clin Nephrol Case Stud 2021 16;9:45-48. Epub 2021 Apr 16.

Pediatric Nephrology, Dialysis, and Transplantation Department of Woman and Child Health, University of Padova, Padova.

A 3-year-old girl came to our attention for fever and upper respiratory tract infection associated with thrombocytopenia, non-immune hemolytic anemia, and acute kidney injury (AKI). Complete blood count and renal function slowly normalized, with no need for dialysis. She was always normotensive with valid diuresis; her neurological status also rapidly improved. Read More

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Therapeutic Plasma Exchange Application in Children Requires Individual Decision.

J Pediatr Intensive Care 2021 Jun 20;10(2):106-109. Epub 2020 Jul 20.

Department of Pediatric Intensive Care, Faculty of Medicine, Istanbul University, Istanbul, Turkey.

Therapeutic plasma exchange (TPE) is a treatment administered with the aim of removing a pathogenic material or compound causing morbidity in a variety of neurologic, hematologic, renal, and autoimmune diseases. In this study, we aimed to assess the indications, efficacy, reliability, complications, and treatment response of pediatric patients for TPE. This retrospective study analyzed data from 39 patients aged from 0 to 18 years who underwent a total of 172 TPE sessions from January 2015 to April 2018 in a tertiary pediatric intensive care unit. Read More

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Identification, Shiga toxin subtypes and prevalence of minor serogroups of Shiga toxin-producing Escherichia coli in feedlot cattle feces.

Sci Rep 2021 Apr 21;11(1):8601. Epub 2021 Apr 21.

Department of Diagnostic Medicine/Pathobiology, Kansas State University, Manhattan, KS, 66506, USA.

Shiga toxin-producing Escherichia coli (STEC) are foodborne pathogens that cause illnesses in humans ranging from mild to hemorrhagic enteritis with complications of hemolytic uremic syndrome and even death. Cattle are a major reservoir of STEC, which reside in the hindgut and are shed in the feces, a major source of food and water contaminations. Seven serogroups, O26, O45, O103, O111, O121, O145 and O157, called 'top-7', are responsible for the majority of human STEC infections in North America. Read More

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A patient with a homozygous diacylglycerol kinase epsilon (DGKE) gene mutation with atypical haemolytic uraemic syndrome and low C3 responded well to eculizumab: a case report.

BMC Nephrol 2021 Apr 20;22(1):140. Epub 2021 Apr 20.

Department Paediatric Nephrology, King Abdullah Specialists Children Hospital, Riyadh, Saudi Arabia.

Background: Atypical haemolytic uraemic syndrome (aHUS) is a rare systemic syndrome characterized by non-immune haemolytic anaemia, thrombocytopenia, and kidney injury. In most cases, alternative complement pathway dysregulation is the identifying cause. Recently, other genetic causes have been identified, including a mutation in the diacylglycerol kinase epsilon (DGKE) gene, which theoretically affect the coagulation pathway and does not affect the complement pathway. Read More

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Clinical and Genetic Characteristics of Atypical Hemolytic Uremic Syndrome in Children: A Chinese Cohort Study.

Nephron 2021 Apr 19:1-13. Epub 2021 Apr 19.

Department of Nephrology, Beijing Children's Hospital, Capital Medical University, Beijing, China.

Background: Atypical hemolytic uremic syndrome (aHUS) is a rare but critical illness. To this date, few studies have reported on the disease in Chinese children.

Methods: We studied a Chinese pediatric cohort to delineate the clinical characteristics, genotypes, and prognosis. Read More

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Correction to: Ravulizumab: A Review in Atypical Haemolytic Uraemic Syndrome.

Yahiya Y Syed

Drugs 2021 Apr;81(6):737

Springer Nature, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

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Prophylactic or Early Use of Eculizumab and Graft Survival in Kidney Transplant Recipients With Atypical Hemolytic Uremic Syndrome in the United States: Research Letter.

Can J Kidney Health Dis 2021 31;8:20543581211003763. Epub 2021 Mar 31.

Nephrology Service, Walter Reed National Military Medical Center, Bethesda, MD, USA.

Introduction: Among kidney transplant recipients (KTRs) with end-stage kidney disease (ESKD) due to atypical hemolytic uremic syndrome (aHUS), recurrence is associated with poor allograft outcomes. We compared graft and patient survival of aHUS KTRs with and without prophylactic/early use of eculizumab, a monoclonal antibody that binds complement protein C5, at the time of transplantation.

Methods: We conducted a retrospective cohort study using the United States Renal Data System. Read More

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Epidemiological investigation of recurrent outbreaks of haemolytic uraemic syndrome caused by Shiga toxin-producing serotype O55:H7 in England, 2014-2018.

Epidemiol Infect 2021 Apr 19;149:e108. Epub 2021 Apr 19.

Field Services, National Infection Service, Public Health England, London, UK.

Recurrent outbreaks of haemolytic uraemic syndrome (HUS) caused by Shiga toxin-producing Escherichia coli (STEC) serotype O55:H7 occurred in England between 2014 and 2018. We reviewed the epidemiological evidence to identify potential source(s) and transmission routes of the pathogen, and to assess the on-going risk to public health. Over the 5-year period, there were 43 confirmed and three probable cases of STEC O55:H7. Read More

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