atypical hemolytic uremic syndrome (aHUS)

URI: https://gptkb.org/entity/atypical_hemolytic_uremic_syndrome_(aHUS)
GPTKB entity
Statements (41)
Predicate Object
gptkbp:instanceOf gptkb:disease
gptkbp:abbreviation gptkb:aHUS
gptkbp:affectsOrgan gptkb:kidney
blood
gptkbp:associatedWith gptkb:CD46_gene
C3 gene
CFB gene
CFH gene
CFI gene
DGKE gene
THBD gene
gptkbp:causedBy genetic disorder
autoantibodies
complement system dysregulation
gptkbp:complication hypertension
neurological symptoms
end-stage renal disease
gptkbp:diagnosedBy blood test
genetic testing
kidney biopsy
gptkbp:distinctFrom gptkb:typical_hemolytic_uremic_syndrome
gptkbp:hasOrphanetID ORPHA:328
gptkbp:hasSubgroup gptkb:thrombotic_microangiopathy
https://www.w3.org/2000/01/rdf-schema#label atypical hemolytic uremic syndrome (aHUS)
gptkbp:ICD-10_code D59.3
gptkbp:inheritance autosomal dominant
autosomal recessive
gptkbp:isNotCausedBy gptkb:Shiga_toxin
gptkbp:mortalityRate high without treatment
gptkbp:OMIM 235400
gptkbp:onset childhood
adulthood
gptkbp:prevalence rare
gptkbp:symptom gptkb:anemia
thrombocytopenia
acute kidney injury
gptkbp:treatment gptkb:eculizumab
gptkb:ravulizumab
plasma exchange
gptkbp:bfsParent gptkb:CFHR4
gptkbp:bfsLayer 8