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Original Articles
- Chronic Sclerosing Hyaline Change and Fatty Metamorphosis Resembling Alcoholic Liver Diseas in Prader-Willi Syndrome.
- Sun Hee Sung, Dong Won Min, Chan Il Park, Ki Sup Chung
- Korean J Pathol. 1993;27(4):407-410.
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Abstract
PDF - A complex syndrome, later called as Prader-Willi syndrome, was first described in 1956 by Prader et al, and Zellweger and Schneider characterized this syndrome as hypogonadism, hypotonia, hypomentia and boesty. It is not rare in western countries and more than 400 cases have been reported until 1983. But our interest arose because of our recent experience of diffuse noncirrhotic fibrosis of the liver in a 6 year-old boy who had the clinical features of Prader-Willi syndrome. The core of liver showed destruction of most of the hepatic lobules, particularly of the acinar zone 3, and replacement bt diffuse fibrosis. The remaining liver cells underwent fatty change, and the overall changes resembled chronic sclerosing hyaline disease of the alcoholic type. Inflammation was negligible. This particular case suggests that the severe fatty change of liver could result in irreversible damage to the hepatocytes and progressive fibrosis.
- Histologic Pattern of Alcoholic Liver Disease in Korea.
- Chan Il Park, Ho Guen Kim, So Young Jin, Mi Kyung Lee, Yoo Bock Lee
- Korean J Pathol. 1989;23(3):292-304.
- 1,686 View
- 21 Download
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Abstract
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- To elucidate the histologic pattern of alcoholic liver disease (ALD) in Korea, liver biopsies from 173 chronic alcoholics with clinical liver diseases were classified according to the pathologic parameters. One hundred and seventeen cases, the sum of 91 of 116 serum HBsAg negative and 26 of 57 HBsAg positive patients, had the histologic evidence of ALD. Fatty change(23.9%), alcoholic fibrosis (AF)(23.1%) and cirrhosis (23.1%), comprised the three major ALDs, and only 8.5% of cases fit the criteria of alcoholic hepatitis. Chronic sclerosing hyaline disease (CSHD), chronic active alcoholic hepatitis (CAAH) and AF, where non-cirrhotic fibrosis is the predominant change, comprised 44.5% of ALD. Both features of ALD and HBV liver disease (HBV-LD) were found in 17 cases that included 8 AF and 7 cirrhosis. These 17 patients tended to consume less alcohol than patients with other types of pure ALD except alcoholic heaptitis. Patients with the serum HBsAg positive ALD (37.4years) were about 8 years younger than those with the serum HBsAg negative ALD (45.1years). More or less fatty change and foamy degeneration were seen in 77.4% and 31.6% of ALD respectively. Mallory bodies, megamitochondria, iron deposition and perihepatocellular fibrosis were found in 20.5%, 29.9%, 42.7% and 77.8%, respectively. These findings indicate that non-cirrhotic chronic ALD such as CSHD, CAAH and AF are the important histologic patterns of ALD in Korea, and that chronic alcohol consumption and HBV may act synergistically in developing liver disease.
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