Genetics and outcome of atypical hemolytic-uremic syndrome in Turkish children: a retrospective study between 2010 and 2017, a single-center experience.

CONCLUSIONS: Morbidity rate is higher in children with aHUS. The renal prognosis and morbidity rate is higher in children with CFH mutations than other children with aHUS. Poor prognosis in aHUS children with CFH mutation depends on the genetic background. PMID: 31705748 [PubMed - in process]
Source: Iranian Journal of Kidney Diseases - Category: Urology & Nephrology Tags: Iran J Kidney Dis Source Type: research

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Publication date: September 2019Source: Advances in Chronic Kidney Disease, Volume 26, Issue 5Author(s): Elizabeth S. Kotzen, Sanjeet Roy, Koyal JainAntiphospholipid syndrome (APS) and other causes of thrombotic microangiopathy (TMA) negatively impact the renal outcomes of patients with systemic lupus erythematosus (SLE) and lupus nephritis. Here we review the diagnosis and management of occlusive renal vascular lesions due to APS and other TMAs, with a focus on patients with SLE and lupus nephritis. The presence of a thrombotic event, unexplained hypertension, thrombocytopenia, or hemolytic anemia should prompt considerat...
Source: Advances in Chronic Kidney Disease - Category: Urology & Nephrology Source Type: research
;oz A Abstract The prospective Chronic Kidney Disease in Children cohort with data collected from 2003 to 2018 provided the first opportunity to characterize the incidence of renal replacement therapy (RRT) initiation over the life course of pediatric kidney diseases. Parametric generalized gamma models were fit and extrapolated for overall RRT, and by specific modality (dialysis or preemptive transplant). Children were stratified by diagnosis: n=650 with non-glomerular (mostly congenital), n=49 glomerular-hemolytic uremic syndrome, and n=216 with glomerular-non-HUS (heterogeneous childhood onset). Estimated years...
Source: Am J Epidemiol - Category: Epidemiology Authors: Tags: Am J Epidemiol Source Type: research
Authors: Chen SF, Chen M Abstract Chronic kidney disease (CKD) is a public health problem worldwide, with increasing incidence and prevalence. The mechanisms underlying the progression to end-stage renal disease (ESRD) is not fully understood. The complement system was traditionally regarded as an important part of innate immunity required for host protection against infection and for maintaining host hemostasis. However, compelling evidence from both clinical and experimental studies has strongly incriminated complement activation as a pivotal pathogenic mediator of the development of multiple renal diseases and p...
Source: Advances in Experimental Medicine and Biology - Category: Research Tags: Adv Exp Med Biol Source Type: research
Angiopoietins (Angs) are a family of vascular growth factors that share multiple cellular functions related to cell survival, proliferation, and migration. Angs play physiological and pathological roles through the Tie tyrosine kinase receptors. The Ang ‐Tie signaling pathway participates in the developmental and tumor‐induced angiogenesis and is also involved in many disease settings, such as vascular diseases, systemic inflammation, and cancers. Since Angs are widely expressed in the kidney, an enormous amount of research focuses on their rol es in the kidney. In this review, we describe the biological functions of t...
Source: FEBS Letters - Category: Biochemistry Authors: Tags: Review Article Source Type: research
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Source: FEBS Letters - Category: Biochemistry Authors: Tags: Review Source Type: research
INTRODUCTION: Atypical hemolytic uremic syndrome (aHUS) is a complement mediated thrombotic microangiopathy that predominantly affects the kidneys although extra-renal manifestations are common. In the pre-eculizumab era, 40-65% of patients either died or had end stage renal disease (ESRD) at 1 year. Long-term renal and cardiovascular outcomes are less well described in the eculizumab era. We conducted this cohort study to describe the renal and cardiovascular outcomes of adult survivors of aHUS, both on and off continued eculizumab therapy.METHODS: Patients with aHUS were identified from the prospective Complement Associa...
Source: Blood - Category: Hematology Authors: Tags: 311. Disorders of Platelet Number or Function: Poster III Source Type: research
Primary glomerular diseases and congenital anomalies of the kidney and urinary tract are the most common causes of kidney disease in children. Although the short-term prognosis is favorable after clinical remission of nephrotic syndrome or successful surgical intervention for congenital anomalies, few studies have evaluated the implications of these and other childhood kidney diseases on the long-term risk of end-stage kidney disease (ESKD). Previous studies have suggested an increased risk of chronic kidney disease (CKD) or kidney function decline in children with a single functioning kidney, children who recover from acu...
Source: Kidney International - Category: Urology & Nephrology Authors: Tags: Nephrology Digest Source Type: research
Authors: Kim SH, Kim HY, Kim SY Abstract Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is mainly plasmapheresis or plasma infusion; however, many children with aHUS will progress to chronic kidney disease despite plasma the...
Source: Korean Journal of Pediatrics - Category: Pediatrics Tags: Korean J Pediatr Source Type: research
ConclusionThis study clarified differences in the relative proportions of primary diseases between patients from Japan and North America and Europe. The difference may be attributable to the lower estimated incidence of STEC-HUS in Japan.
Source: Clinical and Experimental Nephrology - Category: Urology & Nephrology Source Type: research
Purpose of review With improving short-term kidney transplant outcomes, recurrent glomerular disease is being increasingly recognized as an important cause of chronic allograft failure. Further understanding of the risks and pathogenesis of recurrent glomerular disease enable informed transplant decisions, along with the development of preventive and treatment strategies. Recent findings Multiple observational studies have highlighted differences in rates and outcomes for various recurrent glomerular diseases, although these rates have not markedly improved over the last decade. Emerging evidence supports use of ritux...
Source: Current Opinion in Nephrology and Hypertension - Category: Urology & Nephrology Tags: DIALYSIS AND TRANSPLANTATION: Edited by J. Kevin Tucker and Anil Chandraker Source Type: research
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