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
'-Deoxy-" '-Difluorocytidine
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