Hämolytisch-urämisches Syndrom im Kindes- und Jugendalter

Hemolytic uremic syndrome (HUS) is defined by the combination of microangiopathic hemolytic anemia, thrombocytopenia and acute functional impairment of the kidneys. The most common form in childhood is STEC-HUS caused by an infection with shiga toxin-producing bacteria leading to hemorrhagic enterocolitis. This results in HUS in approximately 10% of cases. Extrarenal symptoms might occur. The confirmation of the diagnosis is carried out by stool and serological diagnostics. There is no specific therapy available. Complement-mediated HUS gives rise to mutations, antibodies and trigger events, which lead to dysregulation of alternative complement pathways. Due to the specific treatment options with the C5 inhibitor eculizumab the prognosis has significantly improved. In rare cases HUS is caused by infections (e. g. Streptococcus pneumoniae) or other underlying conditions (e. g. cobalamin C deficiency). Due to the complexity of the diagnostics and therapy, treatment should be performed in a specialized pediatric nephrology center.