cholestasis; Cholestasis-lymphedema syndromes; Cholestasis-lymphoedema syndrome; hereditary of Norwegian type
HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Autosomal recessive hereditary intrahepatic cholestasis occurring already in the neonatal period with later development of lymphedema.
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EtiopathogenesisThis section has been translated automatically.
Unclear, a pathological formation of lymph vessels in organs such as the liver is being discussed.
Clinical featuresThis section has been translated automatically.
LiteratureThis section has been translated automatically.
- Aagenaes Ø, van der Hagen CB, Refsum S (1968) Hereditary recurrent intrahepatic cholestasis from birth. Arch Dis Child 43: 646
- Aagenaes Ø (1998) Hereditary cholestasis with lymphoedema (Aagenaes syndrome, cholestasis-lymphoedema syndrome). New cases and follow-up from infancy to adult age. Scand J Gastroenterol 33: 335-345
Incoming links (5)Aagenaes, øysteine; Cholestasis; Cholestasis-lymphedema syndromes; Hereditary of norwegian type; Malformation lymphatic;
Outgoing links (1)Lymphedema (overview);
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