Novel homozygous CD46 variant with C‐isoform expression affects C3b inactivation in atypical hemolytic uremic syndrome
Sign Up to like & getrecommendations! Published in 2022 at "European Journal of Immunology"
DOI: 10.1002/eji.202249838
Abstract: Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy that may lead to organ failure. Dysregulation of the complement system can cause aHUS, and various disease‐related variants in the complement regulatory protein CD46 are described.… read more here.
Keywords: atypical hemolytic; expression; uremic syndrome; cd46 ... See more keywords
Eculizumab interruption in atypical hemolytic uremic syndrome due to shortage: analysis of a Brazilian cohort
Sign Up to like & getrecommendations! Published in 2021 at "Journal of Nephrology"
DOI: 10.1007/s40620-020-00920-z
Abstract: Background The risk of eculizumab therapy discontinuation in patients with atypical hemolytic uremic syndrome (aHUS) is unclear. The main objective of this study was to analyze the risk of aHUS relapse after eculizumab interruption due to… read more here.
Keywords: nephrology; atypical hemolytic; relapse; shortage ... See more keywords
Atypical hemolytic uremic syndrome due to DGKE mutation and response to eculizumab: lessons for the clinical nephrologist
Sign Up to like & getrecommendations! Published in 2021 at "Journal of Nephrology"
DOI: 10.1007/s40620-020-00925-8
Abstract: An 18-month-old white boy presented with non-bloody diarrhea and pallor and one week of cough and nasal congestion. On examination, he was hypertensive (115/99) and tachycardic (140 bpm). Pertinent labs included normocytic hemolytic anemia with… read more here.
Keywords: atypical hemolytic; uremic syndrome; hemolytic uremic; eculizumab ... See more keywords
Eculizumab treatment in atypical hemolytic uremic syndrome: correlation between functional complement tests and drug levels
Sign Up to like & getrecommendations! Published in 2022 at "Journal of Nephrology"
DOI: 10.1007/s40620-021-01187-8
Abstract: Atypical hemolytic uremic syndrome (aHUS) is characterized by platelet consumption, hemolysis, and renal injury. Eculizumab, a humanized antibody that blocks complement activity, has been successfully used in aHUS, but the best treatment schedule has not… read more here.
Keywords: atypical hemolytic; drug; hemolytic uremic; treatment ... See more keywords
Severe and malignant hypertension are common in primary atypical hemolytic uremic syndrome.
Sign Up to like & getrecommendations! Published in 2019 at "Kidney international"
DOI: 10.1016/j.kint.2019.05.014
Abstract: Malignant hypertension is listed among the causes of secondary thrombotic microangiopathy (TMA), but pathogenic mutations in complement genes have been reported in patients with hypertension-induced TMA. Here we investigated the frequency and severity of hypertension… read more here.
Keywords: uremic syndrome; hemolytic uremic; atypical hemolytic; malignant hypertension ... See more keywords
Efficacy of Eculizumab Therapy for Atypical Hemolytic Uremic Syndrome Recurrence and Antibody-Mediated Rejection Progress After Renal Transplantation With Preformed Donor-Specific Antibodies: Case Report.
Sign Up to like & getrecommendations! Published in 2017 at "Transplantation proceedings"
DOI: 10.1016/j.transproceed.2016.10.013
Abstract: Atypical hemolytic uremic syndrome (aHUS) develops as the result of unregulated complement progression and precipitates de novo thrombotic microangiopathy. Plasma therapy is used to control the progression of the complement cascade, but that therapy is not… read more here.
Keywords: atypical hemolytic; recurrence; renal transplantation; plasma therapy ... See more keywords
Efficacy and Safety of Eculizumab in Kidney Transplant Patients With Primary Atypical Hemolytic-Uremic Syndrome.
Sign Up to like & getrecommendations! Published in 2021 at "Transplantation proceedings"
DOI: 10.1016/j.transproceed.2021.09.063
Abstract: The atypical hemolytic-uremic syndrome (aHUS) is characterized by the triad of non-immune hemolytic anemia, thrombocytopenia, and acute renal failure. The aHUS is related to complement dysregulation; since the approval of eculizumab for this entity (a… read more here.
Keywords: atypical hemolytic; kidney transplant; hemolytic uremic; transplant patients ... See more keywords
Low efficacy of vaccination against serogroup B meningococci in patients with atypical hemolytic uremic syndrome
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DOI: 10.1042/bsr20200177
Abstract: Abstract Background: The C5 complement inhibitor eculizumab is first-line treatment in atypical hemolytic uremic syndrome (aHUS) going along with a highly increased risk of meningococcal infections. Serogroup B meningococci (MenB) are the most frequently encountered… read more here.
Keywords: uremic syndrome; hemolytic uremic; atypical hemolytic; treatment ... See more keywords
A Guide for Adult Nephrologists and Hematologists to Managing Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy in Teens Transitioning to Young Adults.
Sign Up to like & getrecommendations! Published in 2022 at "Advances in chronic kidney disease"
DOI: 10.1053/j.ackd.2022.04.003
Abstract: Atypical hemolytic uremic syndrome and C3 glomerulopathy/immune complex membranoproliferative glomerulonephritis are ultra-rare chronic, complement-mediated diseases with childhood manifestation in a majority of cases. Transition of clinical care of patients from pediatric to adult nephrologists-typically with… read more here.
Keywords: atypical hemolytic; adult nephrologists; uremic syndrome; syndrome glomerulopathy ... See more keywords
Neonatal Atypical Hemolytic Uremic Syndrome in the Eculizumab Era
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DOI: 10.1055/s-0041-1731057
Abstract: The atypical hemolytic uremic syndrome (aHUS) in the newborn is a rare disease, with high morbidity. Eculizumab, considered a first-line drug in older children, is not approved in neonates and in children weighing less than… read more here.
Keywords: neonatal atypical; atypical hemolytic; uremic syndrome; hemolytic uremic ... See more keywords
Quantifying the economic effects of ravulizumab versus eculizumab treatment in patients with atypical hemolytic uremic syndrome
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DOI: 10.1080/13696998.2022.2027706
Abstract: Abstract Aims This study compared the aggregate duration of treatment administration of approved eculizumab and ravulizumab treatment regimens and resultant productivity implications for patients with atypical hemolytic uremic syndrome (aHUS) and their caregivers. Methods The… read more here.
Keywords: ravulizumab 100; atypical hemolytic; treatment; productivity ... See more keywords