Aagenaes syndromeI89.0

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

cholestasis; Cholestasis-lymphedema syndromes; Cholestasis-lymphoedema syndrome; hereditary of Norwegian type

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HistoryThis section has been translated automatically.

Aagenaes, 1968

DefinitionThis section has been translated automatically.

Autosomal recessive hereditary intrahepatic cholestasis occurring already in the neonatal period with later development of lymphedema.

EtiopathogenesisThis section has been translated automatically.

Unclear, a pathological formation of lymph vessels in organs such as the liver is being discussed.

ClinicThis section has been translated automatically.

Cholestasis, lymphedema.

LiteratureThis section has been translated automatically.

  1. Aagenaes Ø, van der Hagen CB, Refsum S (1968) Hereditary recurrent intrahepatic cholestasis from birth. Arch Dis Child 43: 646
  2. 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

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer