Familial heterozygous hypobetalipoproteinemia, extrahepatic primary malignancy, and hepatocellular carcinoma
Familial heterozygous hypobetalipoproteinemia, extrahepatic primary malignancy, and hepatocellular carcinoma
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DOI：
10.1023/a:1026646618643
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发表时间：
1998-11-01
影响因子：
3.1
通讯作者：
Bagni, A
中科院分区：
文献类型：
--
作者：
Lonardo, A;Tarugi, P;Bagni, A
Familial hypobetalipoproteinemia is a disorder of lipoprotein metabolism whose incidence in the heterozygote form is estimated to be about 1 in 500 to 1 in 3000 (1, 2). Many cases of dominantly transmitted hypobetalipoproteinemia are due to truncations of apolipoprote in B (1). In contrast to the severe clinical expression of homozygous hypobetalipoproteinemia and abetalipoproteinemia, most patients with familial heterozygous hypobetalipoproteinemia (FHHBL) are asymptomatic and are most often detected during family screening or population studies (1). The gastroenterological spectrum of its manifestations and complications may include fat malabsorption (3) or transient malabsorption during infancy (4), a tendency to cholelithiasis (5), and perhaps cirrhosis (6). More recently, fatty changes have been reported in the livers of three subjects with FHHBL (7±9) but the natural history of such involvement is presently unknown (9).The male sex, genetic predisposition, personal habits (drinking alcohol and smoking tobacco), hepatitis B and C viral infections, and cirrhosis are thought to play a role in the etiology of hepatocellular carcinoma (HCC) mostly through repeated bouts of hepatocellular necrosis and regeneration (10), but their relative importance
