Aagenaes syndrome | definition of Aagenaes syndrome by Medical dictionary
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an idiopathic form of familial intrahepatic cholestasis associated with lymphedema of the lower extremities.
Aagenaes, Oystein, Norwegian pediatrician, 1925–.
Aagenaes syndrome - inherited condition of lymphedema and arrested bile flow.
References in periodicals archive
Examples of metabolic diseases with hepatic presentation are tyrosinemia, Wilson's disease, progressive familial intrahepatic cholestasis (PFIC), Aagenaes syndrome
, glycogen storage disease, non-alcoholic steatohepatitis (NASH)12-14.
Abigail Cane, aged 11, died from multiple organ failure as a result of Aagenaes syndrome, a condition of the lymph vessels on November 20, 2009 in Birmingham Children's Hospital.
Aagenaes syndrome causes lymphedema of the legs, resulting in swelling, and prevents bile from flowing from the liver to the intestine.