CareLit Fachartikel
Atypical Hemolytic Uremic Syndrome (aHUS): Mutations in components of complement and their impact on the clinical course
N Bachmann, A Melk, B Kranz, H Fehrenbach, N Wirtz, K Häffner, L Pape, C Bergmann · Klinische Pädiatrie · 2011 · Heft 03
Dokument
976264
CareLit-ID
Jahr
2011
Publikation
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Zusammenfassung
Hemolytic uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia, and renal impairment, and is the most common cause of acute renal failure in childhood. Most cases occur secondary to infection with Shiga toxin-producing bacteria. About 10% of HUS patients are classified as atypical (aHUS) with a generally poorer prognosis. Genetic complement abnormalities play a major role in aHUS and lead to uncontrolled overactivation of the alternative complement pathway. While the glomerular vasculature of the kidney is the main target, about 20% of patients additionally show extrarenal involvement (CN…
Schlagworte
atypical hemolytic uremic syndrome (aHUS)
complement system
end stage renal disease (ESRD)
genotype-phenotype correlations
oligogenic inheritance
mutational load theory
Klinische Pädiatrie
