Remission of aHUS neurological damage with eculizumab
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چکیده
منابع مشابه
Remission of aHUS neurological damage with eculizumab
Atypical haemolytic uraemic syndrome (aHUS) is a rare disease characterized by haemolytic microangiopathic anaemia, thrombocytopaenia and acute onset of renal failure, in the absence of Escherichia coli infection. Renal damage usually progresses to end-stage renal disease (ESRD), sometimes being accompanied by signs of extrarenal thrombotic microangiopathy (TMA). We report a case of full neurol...
متن کاملCan eculizumab be discontinued in aHUS?
BACKGROUND The management of atypical hemolytic uremic syndrome (aHUS) has evolved into better control of thrombotic microangiopathy (TMA) and recovery of renal functions since the recent introduction of the terminal complement cascade blocker, eculizumab, into clinical use. Better characterization of genotype-phenotype relations has become possible with genetic and clinical studies. However, t...
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Atypical hemolytic uremic syndrome (aHUS) is a genetic, life-threatening disease characterized by uncontrolled complement activation, systemic thrombotic microangiopathy (TMA), and vital organ damage. We evaluated the effect of terminal complement blockade with the anti-C5 monoclonal antibody eculizumab on biomarkers of cellular processes involved in TMA in patients with aHUS longitudinally, du...
متن کاملSafety and Efficacy of Eculizumab in Pediatric Patients With aHUS, With or Without Baseline Dialysis
• Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by chronic, uncontrolled complement activation and thrombotic microangiopathy (TMA), leading to renal and other end organ damage • Plasma exchange/plasma infusion (PE/PI) provides limited clinical benefit to children with aHUS— 29% progress to end-stage renal disease or die within the first year of diagnosis.1,2 PE/PI is als...
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In typical hemolytic uremic syndrome (HUS) approximately 25% of patients show central nervous system (CNS) involvement often leading to serious long-term disabilities. We used the C5-complement inhibitor Eculizumab as rescue therapy. From 2011 to 2014, 11 children (median age 22 months, range 11-175) with enterohemorrhagic Escherichia coli-positive HUS requiring dialysis who had seizures (11/11...
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ژورنال
عنوان ژورنال: Clinical Kidney Journal
سال: 2015
ISSN: 2048-8505,2048-8513
DOI: 10.1093/ckj/sfu144