Conditions / Syndrome
Aagenaes syndrome
info · Syndrome
A syndrome that is characterized 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.
Signs and symptoms
- Erysipelas
- Malabsorption
- Hyperlipidemia
- Elevated circulating alkaline phosphatase concentration
- Cirrhosis
- Hepatomegaly
- Conjugated hyperbilirubinemia
- Neonatal cholestatic liver disease
- Lymphedema
- Elevated circulating hepatic transaminase concentration
Also known as: Cholestasis-edema syndrome, Norwegian type