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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.
It is also called cholestasis-lymphedema syndrome (CLS).
Medizinwörterbuch für Ạ̈rzte und Krankenschwestern