Aagenaes syndrome


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Aagenaes syndrome

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.
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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.
FREQUENCY OF CAUSES OF CHRONIC LIVER DISEASE IN CHILDREN
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.
GIRL, 11, LOSES HER RARE ILLNESS FIGHT

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