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Atypical hemolytic-uremic syndrome

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Description

Hemolytic-uremic syndrome (HUS) is characterized by hemolytic anemia, thrombocytopenia, and renal failure caused by platelet thrombi in the microcirculation of the kidney and other organs. The onset of atypical HUS (aHUS) ranges from the neonatal period to adulthood. Genetic aHUS accounts for an estimated 60% of all aHUS. Individuals with genetic aHUS frequently experience relapse even after complete recovery following the presenting episode; 60% of genetic aHUS progresses to end-stage renal disease (ESRD).

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  • Mode of Inheritance

    VARIANTS

    70

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    Genes

    External Links

    • OMIM
    • Orphanet

      2134

    • HPO
    • Medgen

      C2931788

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