Chronic Sclerosing Hyaline Change and Fatty Metamorphosis Resembling Alcoholic Liver Diseas in Prader-Willi Syndrome

ABSTRACT

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.