PMC5849380_01
Intense microvesicular steatosis with focal deposition of cholesterol crystals in Kupffer cell at optical mycroscopy.
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PMC5849380_01_0103-0582-rpp-2018-36-1-00016-gf1.jpg
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Liver biopsy was indicated and showed diffuse enlargement of hepatocyte volume with intense microvesicular steatosis and ballooning, as well as triglyceride deposits in Kupffer cells and portal macrophages, associated with occasional deposition of cholesterol crystals (Figure 1).
Triglyceride deposits were sometimes fragmented with irregular striations. ("bitten-apple" aspect).
0103-0582-rpp-2018-36-1-00016-gf2.jpg
PMC5849380_01_0103-0582-rpp-2018-36-1-00016-gf2.jpg
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The ultrastructural findings were consistent with the previous morphological hypothesis of Wolman Disease/CESD (Figure 2).