Metadata | |
---|---|
ID | DOID:6691 |
Name | Aagenaes syndrome |
Definition | A syndrome that is characterized by congenital hypoplasia of lymph vessels, which causes lymphedema of the legs and recurrent cholestasis in infancy, and slow progress to hepatic cirrhosis and giant cell hepatitis with fibrosis of the portal tracts. http://en.wikipedia.org/wiki/Aagenaes_syndrome |
Xrefs |
SNOMEDCT_US_2023_03_01:28724005 |
Subsets |
DO_rare_slim NCIthesaurus |
Synonyms |
Cholestasis-edema syndrome, Norwegian type [EXACT] |
Parent Relationships |
is_a syndrome |