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Ultrastructure of Kupffer cells and hepatocytes in liverbioptate was evaluated in a 17-year-old boy with Dubin-Johnson syndrome(DJS).The liver tissue obtainedby needle biopsy was fixed in glutaraldehyde andparaformaldehyde and routinely processed for electronmicroscopic analysis.The ultrastructural examinations ofliver bioptate revealed the accumulation of membrane-bound,electron-dense lysosomal granules within thecytoplasm of hepatocytes,characteristic of DJS.Theywere located mainly in the vicinity of the biliary pole,andpreferentially in the centrilobular region that correspondedto the pigment deposits seen under light microscope.Thepresence of the granules was accompanied by dilatedelements of the granular endoplasmic reticulum andparacrystalline mitochondrial inclusions as well as dilationof the bile canaliculi.The changes in hepatocytes co-existed with marked stimulation and enhanced phagocyticactivity of Kupffer cells.This was manifested in theaccumulation of pigment deposits within their cytoplasmthat corresponded to those observed in hepatocytes.Hyperactive pericentral Kupffer cells which are involvedin the response to pigmentary material originating fromdisintegrated hepatocytes may play an essential role inthe development of DJS.
Ultrastructure of Kupffer cells and hepatocytes in liver bioptate was evaluated in a 17-year-old boy with Dubin-Johnson syndrome (DJS) .The liver tissue obtained by needle biopsy was fixed in glutaraldehyde andparaformaldehyde and routinely processed for electronmicroscopic analysis.The ultrastructural examinations of liver bioptate revealed the accumulation of membrane-bound, electron-dense lysosomal granules within the cytoplasm of hepatocytes, characteristic of DJS.Theywere located mainly in the vicinity of the biliary pole, andpreferentially in the centrilobular region that correspondedto the pigment deposits seen under light microscope. the granules was accompanied by dilatedelements of the granular endoplasmic reticulum andparacrystalline mitochondrial inclusions as well as dilation of the bile canaliculi. the changes in hepatocytes co-existed with marked stimulation and enhanced phagocyticactivity of Kupffer cells. This was manifested in theaccumulation of pigment deposits wit hin the cytoplasmthat corresponded to those observed in hepatocytes. Hyperactive pericentral Kupffer cells which are involved in the response to pigmentary material originating from established intes for hepatocytes may play an essential role inthe development of DJS.