Term Name: | Aagenaes syndrome |
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Synonyms: | Cholestasis-edema syndrome, Norwegian type |
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. |
Ontology: | Human Disease [DOID:6691] ( DOID:6691 ) |