抄録
A case with glycogen storage disease type I (GSDI) associated with hepatic tumor and severe hypertriglyceridemia was reported. A high density area (1.5×1.9cm) in seg. 8 was revealed by liver CT scan and multiple small fillings with contrast medium appeared in the late phase of the hepatic angiography. Transcatheter hepatic artery embolization (TAE) was performed, since malignancy was highly possible. Following TAE, serum triglyceride (TG) levels decreased transiently and returned to the initial levels after 5 weeks. Depressed liver cell function by TAE might cause a marked depression of the synthesis of TG rich lipoproteins. Post heparin lipolytic activity showed lipoprotein lipase (LPL) activity was extremely low, while hepatic TG lipase was subnormal. These data indicates that the mechanism of hypertriglyceridemia in the patients are combination of the overproduction of TG rich lipoproteins and deficiency of LPL.
