"Hemolytic–uremic syndrome"

Ravulizumab (Ultomiris) - Atypical hemolytic uremic syndrome Return to Article DetailsRavulizumab (Ultomiris)

Ravulizumab (Ultomiris) - Atypical hemolytic uremic syndrome Return to Article DetailsRavulizumab (Ultomiris)

Use of complement C5-inhibitor eculizumab in patients with infection-associated hemolytic uremic syndrome - a case-series report. Hemolytic uremic syndrome (HUS), characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury (AKI), remains a leading cause of pediatric AKI. The complement system has a crucial role in the pathogenesis of atypical hemolytic uremic syndrome (aHUS) and eculizumab (ECZ) was approved as standard of care for its treatment. The two widely characterized forms of infection-associated HUS are Shiga toxin-producing E. coli (STEC)-HUS and Streptococcus pneumoniae-associated (SP)-HUS. Extrarenal manifestations such as central nervous system (CNS) involvement occur approximately in 20% of the cases and are accompanied by higher

The membrane attack complex drives thrombotic microangiopathy in complement mediated atypical hemolytic uremic syndrome. Introduction of complement (C) inhibition into clinical practice has revolutionized the treatment of patients with complement-mediated atypical hemolytic syndrome (aHUS). Our C3 mouse model, engineered around a gain of function point mutation in C3, is associated

Case-Control Study of Factors Associated with Hemolytic Uremic Syndrome among Shiga Toxin-Producing Escherichia coli Patients, Ireland, 2017-2020. Shiga toxin-producing Escherichia coli (STEC) infection can cause potentially fatal hemolytic uremic syndrome (HUS). To determine epidemiologic and bacterial genomic factors associated with HUS, we conducted a retrospective case-control study with 108

Ten tips for managing complement-mediated thrombotic microangiopathies (formerly atypical hemolytic uremic syndrome): narrative review. Complement-mediated thrombotic microangiopathies (CM-TMA) are rare and life-threatening disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ damage. These conditions result from dysregulation of the alternative complement

Guidelines on Hemolytic Uremic Syndrome by Indian Society of Pediatric Nephrology: Key Messages Hemolytic uremic syndrome is an important cause of acute kidney injury that requires dialysis in children. The diagnosis and management is difficult due to limited diagnostic facilities and non-availability of specific complement inhibitors. We describe salient features of the recent Indian Society of Pediatric Nephrology consensus guidelines on hemolytic uremic syndrome.

Hot Spot of Complement Factor I Rare Variant p.Ile357Met in Patients With Hemolytic Uremic Syndrome. Atypical haemolytic uremic syndrome (aHUS) is a rare kidney disease due to a dysregulation of the complement alternative pathway (AP). Complement factor I (CFI) negatively regulates the AP and CFI gene rare variants have been associated to aHUS with a low disease penetrance. We report 10 unrelated

Anti-C5 monoclonal antibody treatment showing pathological resolution of complement-mediated atypical hemolytic uremic syndrome: a case report. No reports have shown histological changes before and after anti-C5 monoclonal antibody treatment in patients with atypical hemolytic uremic syndrome (aHUS). Here, we report a rare case of complement-mediated aHUS with a complement factor H (CFH

A Case-Based Narrative Review of Pregnancy-Associated Atypical Hemolytic Uremic Syndrome/Complement-Mediated Thrombotic Microangiopathy. Atypical hemolytic uremic syndrome (aHUS) is a complement-mediated thrombotic microangiopathy (TMA), caused by uncontrolled activation of the alternative complement pathway in the setting of autoantibodies to or rare pathogenic genetic variants in complement

An expert discussion on the atypical hemolytic uremic syndrome nomenclature-identifying a road map to precision: a report of a National Kidney Foundation Working Group. The term atypical hemolytic uremic syndrome has been in use since the mid-1970s. It was initially used to describe the familial or sporadic form of hemolytic uremic syndrome as opposed to the epidemic, typical form of the disease . Over time, the atypical hemolytic uremic syndrome term has evolved into being used to refer to anything that is not Shiga toxin-associated hemolytic uremic syndrome. The term describes a heterogeneous group of diseases of disparate causes, a circumstance that makes defining disease-specific natural history and/or targeted treatment approaches challenging. A working group of specialty-specific experts

Pearls & Oy-sters: Neurologic Involvement in Shiga Toxin-Associated Hemolytic Uremic Syndrome. Shiga toxin-producing (STEC) is among the most common pathogens that cause bacterial enteritis. They can also lead to extraintestinal manifestations including hemolytic uremic syndrome (HUS), which is defined by the triad of hemolytic anemia, thrombocytopenia, and acute renal dysfunction due to Shiga

Treatment discontinuation in adults with atypical hemolytic uremic syndrome (aHUS): a qualitative study of international experts' perspectives with associated cost-consequence analysis. Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA) related to congenital mutations impeding control of the alternative pathway of complement. Following approval

Assessing the Impact of Prophylactic Eculizumab on Renal Graft Survival in Atypical Hemolytic Uremic Syndrome. Atypical hemolytic uremic syndrome (aHUS) is a rare cause of end-stage kidney disease and associated with poor outcomes after kidney transplantation from early disease recurrence. Prophylactic eculizumab treatment at the time of transplantation is used in selected patients with aHUS. We

Atypical Hemolytic Uremic Syndrome Occurring After Receipt of mRNA-1273 COVID-19 Vaccine Booster: A Case Report. Atypical hemolytic uremic syndrome (aHUS) is a subtype of thrombotic microangiopathy (TMA) characterized by a dysregulation of the alternative complement pathway. Here, we report a previously healthy 38-year-old woman in whom aHUS developed after a COVID-19 vaccine booster. One day

X-linked C1GALT1C1 mutation causes atypical hemolytic uremic syndrome. Hemolytic-uremic syndrome (HUS), mostly secondary to infectious diseases, is a common cause of acute kidney injury in children. It is characterized by progressive acute kidney failure due to severe thrombotic microangiopathy, associated with nonimmune, Coombs-negative hemolytic anemia and thrombocytopenia. HUS is caused mostly

Mutations in Atypical Hemolytic Uremic Syndrome Provide Evidence for the Role of Calcium in Complement Factor I. Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy. Genetic variants in complement proteins are found in ~60% of patients. Of these, ~15% carry mutations in complement Factor I. Factor I (FI) is a multi-domain serine protease that cleaves and thereby

Purtscher-Like Retinopathy Associated With Atypical Hemolytic Uremic Syndrome. This case report discusses a diagnosis of atypical hemolytic uremic syndrome in a woman aged 38 years who presented with progressively blurry vision in both eyes over a period of 10 days.

Sporadic Shiga Toxin-Producing Escherichia coli-Associated Pediatric Hemolytic Uremic Syndrome, France, 2012-2021. Shiga toxin-producing Escherichia coli-associated pediatric hemolytic uremic syndrome (STEC-HUS) remains an important public health risk in France. Cases are primarily sporadic, and geographic heterogeneity has been observed in crude incidence rates. We conducted a retrospective

How I diagnose and treat atypical hemolytic uremic syndrome. Our understanding and management of atypical hemolytic uremic syndrome (aHUS) have dramatically improved in the last decade. aHUS has been established as a prototypic disease resulting from a dysregulation of the complement alternative C3 convertase. Subsequently, prospective nonrandomized studies and retrospective series have shown