| Abstract: | To explore the pathogenesis of hepatic steatosis in severe motor and intellectual disabilities (SMID), we immunohistochemically examined the accumulation of oxidative products in 8 autopsy cases of SMID, 5 of which pathologically showed hepatic steatosis. Three of the 8 cases also demonstrated fibrosis in Glisson's sheath. Serial sections of liver were treated with monoclonal antibodies to oxidative products to proteins, lipids and nucleosides. Immunoreactivity for 4-hydroxynonenal, a marker of oxidative damage to lipids, was increased in the cytoplasm of hepatocytes in 5 cases; 2 with steatosis plus fibrosis, one with only fibrosis, and 2 with only hepatic steatosis. One case showed immunoreactivity for advanced glycation endproduct, a marker of oxidative damage to protein, in hepatocytes. There was no immunoreactivity for 8-hydroxy-2'-deoxyguanosine and hexanoyl lysine, markers of oxidative damage to DNA and lipids, respectively. These findings partly mimic those observed in adult patients with nonalcoholic steatohepatitis. The results suggested the involvement of oxidative damage to lipids in the pathogenesis of hepatic steatosis and/or fibrosis in patients with SMID. |
