A child with atypical hemolytic uremic syndrome: Is there a rationale to stop eculizumab?

A child with atypical hemolytic uremic syndrome: Is there a rationale to stop eculizumab? Clin Nephrol. 2020 Jan 27;: Authors: Kersnik Levart T PMID: 31983381 [PubMed - as supplied by publisher]
Source: Clinical Nephrology - Category: Urology & Nephrology Authors: Tags: Clin Nephrol Source Type: research

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AbstractThrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy caused by severely reduced activity of the von-Willebrand factor-cleaving protease ADAMTS13, mainly caused by anti-ADAMTS-13 antibodies. Although several test systems for ADAMTS13 measurement exist, long turn-around times hamper the usability in daily practice. We performed a method comparison study for two commercially available ADAMTS13 assays and evaluated the agreement between the fully-automated rapid turn-over HemosIL AcuStar ADAMTS13 Activity assay and the manually performed TECHNOZYM ADAMTS-13 Activity assay. Twenty-f...
Source: Journal of Thrombosis and Thrombolysis - Category: Hematology Source Type: research
Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by systemic thrombotic microangiopathy (TMA) reflected by hemolysis, anemia, thrombocytopenia and systemic organ injury. The optimal ma...
Source: BMC Nephrology - Category: Urology & Nephrology Authors: Tags: Case report Source Type: research
Abstract The glomerular filtration barrier is a highly specialized capillary wall comprising fenestrated endothelial cells, podocytes, and an intervening basement membrane. In glomerular disease, this barrier loses functional integrity, allowing the passage of macromolecules and cells, and there are associated changes in both cell morphology and the extracellular matrix. Over the past three decades there has been a transformation in our understanding about glomerular disease, fueled by genetic discovery, and this is leading to exciting advances in our knowledge about glomerular biology and pathophysiology. In curr...
Source: Clinical Journal of the American Society of Nephrology : CJASN - Category: Urology & Nephrology Authors: Tags: Clin J Am Soc Nephrol Source Type: research
Enterohemorrhagic Escherichia coli (EHEC) is a highly pathogenic strain leading to hemorrhagic colitis and to the hemolytic-uremic syndrome (HUS) in humans. The mechanisms by which pathogenic E. coli infect and colonize humans leading to the typical disease pattern are in focus of many investigations. The adhesion of EHEC to epithelial cells by the coordinated translocation of receptor Tir and surface expression of corresponding adhesin intimin is a key event in host–pathogen-interaction. However, less is known about other adhesins encoded by EHEC, especially about the complex set of fimbrial adhesins varying among v...
Source: Frontiers in Microbiology - Category: Microbiology Source Type: research
Thrombocytopenia-associated multiple organ failure is a clinical phenotype encompassing a spectrum of syndromes associated with disseminated microvascular thromboses. Autopsies performed in patients that died with thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, or disseminated intravascular coagulation reveal specific findings that can differentiate these 3 entities. Significant advancements have been made in our understanding of the pathologic mechanisms of these syndromes. Von Willebrand factor and ADAMTS-13 play a central role in thrombotic thrombocytopenic purpura. Shiga toxins and the complement pathwa...
Source: Critical Care Clinics - Category: Intensive Care Authors: Source Type: research
Thrombotic microangiopathy (TMA) syndromes are characterized by the association of hemolytic anemia, thrombocytopenia and organ injury due to arteriolar and capillary thrombosis.
Source: BMC Nephrology - Category: Urology & Nephrology Authors: Tags: Case report Source Type: research
Source: Clinical Epidemiology - Category: Epidemiology Tags: Clinical Epidemiology Source Type: research
The most severe form of pneumococcal disease is invasive pneumococcal disease (IPD), including empyema, sepsis and meningitis. Thomsen-Friedenreich antigen (TA; Gal β1-3GalNAc) activation is known to be a predictor of Streptococcus pneumoniae-associated hemolytic uremic syndrome (Sp-HUS). There have been limited data to correlate TA activation and overall disease severity of IPD in children. The study aimed to prove the positive correlation between TA activati on and disease severity and to demonstrate the trend of TA level during the disease course.
Source: Pediatrics and Neonatology - Category: Perinatology & Neonatology Authors: Tags: Original Article Source Type: research
Ravulizumab is a long-acting C5 inhibitor engineered from eculizumab with increased elimination half-life, allowing an extended dosing interval from two to eight weeks. Here we evaluate the efficacy and safety of ravulizumab in adults with atypical hemolytic uremic syndrome presenting with thrombotic microangiopathy. In this global, phase 3, single arm study in complement inhibitor-na ïve adults (18 years and older) who fulfilled diagnostic criteria for atypical hemolytic uremic syndrome, enrolled patients received ravulizumab through a 26-week initial evaluation period.
Source: Kidney International - Category: Urology & Nephrology Authors: Tags: Clinical Trial Source Type: research
This study reports the use of an O91-specific polysaccharide depolymerase for the treatment of STEC infection in mice. PMID: 32111587 [PubMed - as supplied by publisher]
Source: Applied and Environmental Microbiology - Category: Microbiology Authors: Tags: Appl Environ Microbiol Source Type: research
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