Research Notes on Aagenaes Syndrome
Aagenaes syndrome is characterized by congenital hypoplasia of lymph vessels.1 This causes lymphedema of the legs and recurrent cholestasis in infancy.1 It can also slowly progress to hepatic cirrhosis and giant-cell hepatitis with fibrosis of the portal tracts.1
Fast Facts:
- Named after Oystein Aagenaes, a Norwegian paediatrician.1
- The genetic cause is unknown, but it is autosomal recessively inherited.1
- The condition is particularly frequent in southern Norway, where more than half the cases are reported.1
- A common feature of the condition is a generalized lymphatic anomaly.1
Top Resources on Aagenaes Syndrome
- LymphedemaPeople: Sharp Aagenaes Syndrome
- OrphaNet: Summary for Aagenaes Syndrome
- Live Journal: Syndromes Associated with Lymphedema
- Wikipedia: Aagenaes Syndrome
- WrongDiagnosis: Introduction to Aagenaes Syndrome
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About this WisdomCard
- Contributor: MistyL
- Reviewer: Tara
- Supervised by: The OrganizedWisdom Physician Review Team
- Last update: Nov. 6, 2008
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