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肝糖原小体:移植患者中的毛玻璃样肝细胞。

Liver glycogen bodies: ground-glass hepatocytes in transplanted patients.

作者信息

Bejarano Pablo A, Garcia Monica T, Rodriguez Maria M, Ruiz Phillip, Tzakis Andreas G

机构信息

Department of Pathology, University of Miami School of Medicine, Jackson Memorial Hospital, 1611 NW 12th Ave Holtz Bldg, Room 2042, Miami, FL 33136, USA.

出版信息

Virchows Arch. 2006 Nov;449(5):539-45. doi: 10.1007/s00428-006-0286-2. Epub 2006 Sep 22.

Abstract

Ground-glass hepatocytes have been described in Lafora's disease, fibrinogen deposition, hepatitis B, type IV glycogenosis, and alcohol aversion (cyanamide) therapy. We encountered ground-glass hepatocytes with intracytoplasmic inclusions in four liver biopsies from three transplanted patients who had none of the above-mentioned underlying diseases. One patient was a 4-year-old boy who had a kidney transplant for severe ureterovesical reflux. Patient 2 was a 52-year-old man who had two liver transplants because of hepatitis C. The third patient was a 7-month-old girl who underwent a multivisceral transplant because of necrotizing enterocolitis and liver failure induced by total parenteral nutrition. The patients developed liver abnormalities from 45 days to 4 years after their transplants. The livers showed conspicuous ground-glass hepatocytes in 90% of the children's samples and 30% of the adult liver cells. The cytoplasmic bodies stained strongly for Gomori methenamine-silver; they were positive for periodic acid-Schiff without diastase, but negative after diastase digestion. They were negative for colloidal iron and hepatitis B core and surface antigens. Electron microscopy revealed non-membrane bound aggregates of glycogen. Idiopathic ground-glass hepatocytes occur in transplanted patients and represent accumulation of altered glycogen. However, their clinical significance and cause are not entirely elucidated.

摘要

在拉福拉病、纤维蛋白原沉积、乙型肝炎、IV型糖原贮积症以及戒酒硫(氰胺)治疗中均有磨玻璃样肝细胞的相关描述。我们在3例移植患者的4次肝活检中发现了伴有胞浆内包涵体的磨玻璃样肝细胞,而这些患者均无上述基础疾病。患者1是一名4岁男孩,因严重输尿管膀胱反流接受了肾移植。患者2是一名52岁男性,因丙型肝炎接受了两次肝移植。第3例患者是一名7个月大的女孩,因坏死性小肠结肠炎和全胃肠外营养引起的肝衰竭接受了多脏器移植。这些患者在移植后45天至4年出现肝脏异常。在儿童样本中,90%的肝脏显示有明显的磨玻璃样肝细胞,在成人肝细胞中这一比例为30%。胞浆小体对Gomori六胺银染色呈强阳性;对高碘酸希夫染色呈阳性(不加淀粉酶),但经淀粉酶消化后呈阴性。对胶体铁以及乙型肝炎核心抗原和表面抗原检测均为阴性。电子显微镜检查显示糖原呈无膜结合的聚集体。特发性磨玻璃样肝细胞见于移植患者,代表了异常糖原的蓄积。然而,其临床意义和病因尚未完全阐明。

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