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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

GARD:370

MESH:C535330

NCI:C35709

OMIM:214900

ORDO:1414

SNOMEDCT_US_2023_03_01:28724005

UMLS_CUI:C0268314

Subsets

DO_rare_slim

NCIthesaurus

Synonyms

Cholestasis-edema syndrome, Norwegian type [EXACT]

Parent Relationships

is_a syndrome

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