Preferred Name |
Atypical hemolytic uremic syndrome |
ID |
http://www.orpha.net/ORDO/Orphanet_2134 |
alternative_term |
aHUS Atypical HUS |
definition |
A rare, genetic thrombotic microangiopathy due to dysregulation of the alternative complement pathway and characterized by the triad of hemolytic anemia, thrombocytopenia, and acute renal dysfunction. |
definition_citation |
Orphanet |
expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=2134 |
has_age_of_onset |
All ages |
has_inheritance |
Autosomal recessive Not applicable Autosomal dominant |
hasDbXref |
OMIM:612926 OMIM:612924 OMIM:612922 MeSH:C538266 ICD-10:D59.3 OMIM:615008 OMIM:609814 UMLS:C2931788 OMIM:612925 OMIM:235400 ICD-11:3A10.Y OMIM:612923 |
label |
Atypical hemolytic uremic syndrome |
notation |
ORPHA:2134 |
part_of | |
prefixIRI |
ORDO:Orphanet_2134 |
prefLabel |
Atypical hemolytic uremic syndrome |
present_in |
Europe AND has_point_prevalence_average_value : 1.0 AND has_point_prevalence_range : 1-9 / 100 000 United States AND has_annual_incidence_average_value : 0.2 AND has_annual_incidence_range : 1-9 / 1 000 000 China AND has_point_prevalence_average_value : 0.0278 AND has_point_prevalence_range : <1 / 1 000 000 |
treeView | |
subClassOf |