Aagenaes syndrome

Type: Term Definitions: 1. an idiopathic form of familial intrahepatic cholestasis associated with lymphedema of the lower extremities.
Found on http://www.medilexicon.com/medicaldictionary.php?t=87615

Aagenaes syndrome

Aagenaes syndrome is a syndrome characterised 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. The genetic cause is unknown, but it is autosomal recessively inherited and the gene i...
Found on http://en.wikipedia.org/wiki/Aagenaes_syndrome
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