Ultrastructure of Kupffer cells and hepatocytes in the Dubin-Johnson syndrome:A case report

在肝 bioptate 的 Kupffer 房间和 hepatocytes 的超微结构与 Dubin-Johnson 症候群(DJS ) 在一个 17 岁的男孩被评估。针活体检视获得的肝织物在 glutaraldehyde 和帕拉甲醛被修理并且习惯性地为电子处理了显微镜的分析。肝 bioptate 的极端结构的考试揭示了膜界限的累积，在 hepatocytes 的细胞质以内的电子稠密的 lysosomal 小粒， DJS 特征。他们主要位于胆汁的杆的附近，并且优先地，在小叶中心区域，那对应于在轻显微镜下面看见的颜料存款。小粒的存在被无粒内质网和帕拉的扩大元素伴随水晶的 mitochondrial 包括以及胆汁 canaliculi 的膨胀。在 hepatocytes 的变化与显著刺激共存了并且提高了 Kupffer 房间的吞噬细胞的活动。这在他们对应于在 hepatocytes 观察的那些的细胞质以内在颜料存款的累积被表明。涉及对从瓦解的 hepatocytes 发源的颜料的材料的反应的中央 Kupffer 房间可以玩的活动过度的仙子在 DJS 的发展的一个必要角色。

Ultrastructure of Kupffer cells and hepatocytes in liver bioptate was evaluated in a 17-year-old boy with Dubin -Johnson syndrome （D.1S）. The liver tissue obtained by needle biopsy was fixed in glutaraldehyde and paraformaldehyde and routinely processed for electron microscopic analysis. The ultrastructural examinations of liver bioptate revealed the accumulation of membranebound, electron-dense lysosomal granules within the cytoplasm of hepatocytes, characteristic of D.1S. They were located mainly in the vicinity of the biliary pole, and preferentially in the centrilobular region that corresponded to the pigment deposits seen under light microscope. The presence of the granules was accompanied by dilated elements of the granular endoplasmic reticulum and paracrystalline mitochondrial inclusions as well as dilation of the bile canaliculi. The changes in hepatocytes coexisted with marked stimulation and enhanced phagocytic activity of Kupffer cells. This was manifested in the accumulation of pigment deposits within their cytoplasm that corresponded to those observed in hepatocytes. Hyperactive pericentral Kupffer cells which are involved in the response to pigmentary material originating from disintegrated hepatocytes may play an essential role in the development of DJS.