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Familial hypobe talipoprote inemia is a disorde r of lipoprote in 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 hypobe talipoprote inemia are due to truncations of apolipoprote in B (1). In contrast to the severe clinical expre ssion of homozygous hypobe talipoprote inemia and abetalipoprote inemia, most patients with familial heterozygous hypobe talipoprote inemia (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 chole lithiasis (5), and perhaps cirrhosis (6). More recently, fatty change s have been reported in the live rs of three subje cts 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 hepatoce llular carcinoma (HCC) mostly through repeated bouts of hepatocellular necrosis and regeneration (10), but their relative importance is dif® cult to ascertain in the individual patient with multiple such risk factors. Conversely, the absence of some of the most common risk factors could highlight epidemiological features of possible etiopathoge netic signi® cance. Some reports have accordingly focused on HCC in noncirrhotic patients from Japan and Europe (11± 13). Similarly extrahepatic primary malignancies (EHPM), the nosological entity including cancers outside the liver in patients with primary liver cancers, are a subject of continuing interest because etiologic factors contributing to the development of malignant neoplasms may possibly become apparent when patients with multiple tumors are compared with those with a single or no tumors (14). Here we report on the ® rst case of HCC associate d with tonsil cancer in a subject with FHHBL due to a truncated form of apolipoprote in (Apo) B (Medline 1983± June 1997) . |
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