Aagenaes syndrome

Notes
  • This is a rare congenital hypoplasia of lymph vessels that causes lymphedema of the legs and recurrent cholestasis in infancy.
  • Mainly found in Norway.
Symptoms
  • Lymphedema of the legs
  • Recurrent cholestasis in infancy
  • Jaundice
  • Hepatomegaly
  • Insufficient bile secretion
  • Underdeveloped lymph vessels
Management
  • Treatment of lymphedema; dietary consideration; low fat diet with fat soluble vitamin supplements; liver transplant.
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