Gilbert综合征肝活检的超微结构特征

Ultrastructural Characteristics in Liver Biopsy of Gilbert's Syndrome

目的:确定Gilbert综合征患者肝组织的超微结构特征,为Gilbert综合征的诊断和鉴别诊断提供新的方法。方法:按电镜常规进行标本制备,应用透射电镜对20例Gilbert综合征患者肝穿刺活检组织进行超微结构观察。结果:肝细胞可出现巨大线粒体,常含有副晶格样包涵体、较明显的基质致密颗粒。肝细胞常见脂褐素颗粒增多,多分布于毛细胆管周围肝细胞内。可出现较有特征性的色素颗粒,大小不等,卵圆形或不规则形,含有电子致密块状颗粒,与电子密度略低的聚集物以及脂滴互相混杂。这些溶酶体颗粒的基质由细小的、弱嗜锇性的颗粒组成。少数颗粒类似Dubin-Johnson综合征的颗粒。但颗粒较小,缺少致密核芯结构。结论:特征性的含粗大电子致密物的溶酶体对Gilbert综合征的诊断有重要参考价值。

Objective： To investigate the ultrastructural characteristics in liver biopsy of Gilbert＇s syndrome and to provide a new method for diagnosis and differential diagnosis of Gilbert＇s syndrome. Methods： The liver biopsy specimens were obtained from 20 pa- tients with Gilbert＇s syndrome and regularly processed for transmission electron microscopy. Results： Giant mitochrondria often accom- panied with paracrystalline inclusions was presented, and numerous matrix dense granules could be found in hepatocytes. An increase in lipofusin granules in hepatocytes was a common finding and appeared in the periphery of bile canaliculus. The specific pigment granules with variable size, oval or irregular shaped, and contained electron dense masses of particles that mixed with less dense aggregates and lipid droplets could also be found. The matrix of these lysosomes was formed by fine, less osmiophilic particles. A few granules may re- semble those found in Dubin-Johnson syndrome. However, these granules were small in size and dense-cores are absent in therrL Conclusion： The specific lysosomes containing electron dense particles in hepatocytes is of great value for diagnosis of Gilbert＇s syndrome.