FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W2158943356> ?p ?o ?g. }

• W2158943356 endingPage "60" @default.
• W2158943356 startingPage "60" @default.
• W2158943356 abstract "Hemolytic uremic syndrome (HUS) is defined by the triad of mechanical hemolytic anemia, thrombocytopenia and renal impairment. Atypical HUS (aHUS) defines non Shiga-toxin-HUS and even if some authors include secondary aHUS due to Streptococcus pneumoniae or other causes, aHUS designates a primary disease due to a disorder in complement alternative pathway regulation. Atypical HUS represents 5 -10% of HUS in children, but the majority of HUS in adults. The incidence of complement-aHUS is not known precisely. However, more than 1000 aHUS patients investigated for complement abnormalities have been reported. Onset is from the neonatal period to the adult age. Most patients present with hemolytic anemia, thrombocytopenia and renal failure and 20% have extra renal manifestations. Two to 10% die and one third progress to end-stage renal failure at first episode. Half of patients have relapses. Mutations in the genes encoding complement regulatory proteins factor H, membrane cofactor protein (MCP), factor I or thrombomodulin have been demonstrated in 20-30%, 5-15%, 4-10% and 3-5% of patients respectively, and mutations in the genes of C3 convertase proteins, C3 and factor B, in 2-10% and 1-4%. In addition, 6-10% of patients have anti-factor H antibodies. Diagnosis of aHUS relies on 1) No associated disease 2) No criteria for Shigatoxin-HUS (stool culture and PCR for Shiga-toxins; serology for anti-lipopolysaccharides antibodies) 3) No criteria for thrombotic thrombocytopenic purpura (serum ADAMTS 13 activity > 10%). Investigation of the complement system is required (C3, C4, factor H and factor I plasma concentration, MCP expression on leukocytes and anti-factor H antibodies; genetic screening to identify risk factors). The disease is familial in approximately 20% of pedigrees, with an autosomal recessive or dominant mode of transmission. As penetrance of the disease is 50%, genetic counseling is difficult. Plasmatherapy has been first line treatment until presently, without unquestionable demonstration of efficiency. There is a high risk of post-transplant recurrence, except in MCP-HUS. Case reports and two phase II trials show an impressive efficacy of the complement C5 blocker eculizumab, suggesting it will be the next standard of care. Except for patients treated by intensive plasmatherapy or eculizumab, the worst prognosis is in factor H-HUS, as mortality can reach 20% and 50% of survivors do not recover renal function. Half of factor I-HUS progress to end-stage renal failure. Conversely, most patients with MCP-HUS have preserved renal function. Anti-factor H antibodies-HUS has favourable outcome if treated early." @default.
• W2158943356 created "2016-06-24" @default.
• W2158943356 creator A5001037667 @default.
• W2158943356 creator A5025683973 @default.
• W2158943356 date "2011-01-01" @default.
• W2158943356 modified "2023-10-17" @default.
• W2158943356 title "Atypical hemolytic uremic syndrome" @default.
• W2158943356 cites W1591524787 @default.
• W2158943356 cites W1649964539 @default.
• W2158943356 cites W1686008017 @default.
• W2158943356 cites W1915290779 @default.
• W2158943356 cites W1965005024 @default.
• W2158943356 cites W1966590389 @default.
• W2158943356 cites W1967273664 @default.
• W2158943356 cites W1969502372 @default.
• W2158943356 cites W1970432269 @default.
• W2158943356 cites W1971052994 @default.
• W2158943356 cites W1973644206 @default.
• W2158943356 cites W1974010028 @default.
• W2158943356 cites W1976694239 @default.
• W2158943356 cites W1977035416 @default.
• W2158943356 cites W1979317819 @default.
• W2158943356 cites W1979835176 @default.
• W2158943356 cites W1979915137 @default.
• W2158943356 cites W1981278530 @default.
• W2158943356 cites W1982517328 @default.
• W2158943356 cites W1983899909 @default.
• W2158943356 cites W1991598211 @default.
• W2158943356 cites W1994622828 @default.
• W2158943356 cites W1996708969 @default.
• W2158943356 cites W1998930515 @default.
• W2158943356 cites W1999827339 @default.
• W2158943356 cites W1999997207 @default.
• W2158943356 cites W2004415398 @default.
• W2158943356 cites W2005969028 @default.
• W2158943356 cites W2006813617 @default.
• W2158943356 cites W2008607429 @default.
• W2158943356 cites W2010753535 @default.
• W2158943356 cites W2011330983 @default.
• W2158943356 cites W2011726627 @default.
• W2158943356 cites W2012403186 @default.
• W2158943356 cites W2012492081 @default.
• W2158943356 cites W2012630248 @default.
• W2158943356 cites W2013867475 @default.
• W2158943356 cites W2016660261 @default.
• W2158943356 cites W2016859455 @default.
• W2158943356 cites W2017183118 @default.
• W2158943356 cites W2020016942 @default.
• W2158943356 cites W2021691030 @default.
• W2158943356 cites W2024743936 @default.
• W2158943356 cites W2027086946 @default.
• W2158943356 cites W2028031291 @default.
• W2158943356 cites W2030337128 @default.
• W2158943356 cites W2031109264 @default.
• W2158943356 cites W2031628865 @default.
• W2158943356 cites W2035128042 @default.
• W2158943356 cites W2035432263 @default.
• W2158943356 cites W2036607489 @default.
• W2158943356 cites W2036694775 @default.
• W2158943356 cites W2037516600 @default.
• W2158943356 cites W2039756163 @default.
• W2158943356 cites W2042777883 @default.
• W2158943356 cites W2044200772 @default.
• W2158943356 cites W2046192010 @default.
• W2158943356 cites W2046363187 @default.
• W2158943356 cites W2046369602 @default.
• W2158943356 cites W2048117130 @default.
• W2158943356 cites W2051293525 @default.
• W2158943356 cites W2055923362 @default.
• W2158943356 cites W2057270534 @default.
• W2158943356 cites W2057724980 @default.
• W2158943356 cites W2059727759 @default.
• W2158943356 cites W2060435116 @default.
• W2158943356 cites W2061639439 @default.
• W2158943356 cites W2062115674 @default.
• W2158943356 cites W2062890990 @default.
• W2158943356 cites W2064081783 @default.
• W2158943356 cites W2066302161 @default.
• W2158943356 cites W2067053006 @default.
• W2158943356 cites W2069574895 @default.
• W2158943356 cites W2070017727 @default.
• W2158943356 cites W2071004236 @default.
• W2158943356 cites W2071090370 @default.
• W2158943356 cites W2073551050 @default.
• W2158943356 cites W2073579251 @default.
• W2158943356 cites W2074988635 @default.
• W2158943356 cites W2075460468 @default.
• W2158943356 cites W2075818946 @default.
• W2158943356 cites W2078820067 @default.
• W2158943356 cites W2079142360 @default.
• W2158943356 cites W2087564804 @default.
• W2158943356 cites W2089013072 @default.
• W2158943356 cites W2094727724 @default.
• W2158943356 cites W2097173937 @default.
• W2158943356 cites W2098315002 @default.
• W2158943356 cites W2098383030 @default.
• W2158943356 cites W2104082467 @default.
• W2158943356 cites W2106080176 @default.

• next