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地中海贫血所致肾含铁血黄素沉着症:一项结合电子探针X射线微量分析的光镜和电镜研究

Renal hemosiderosis due to thalassemia: a light and electron microscopy study with electron probe X-ray microanalysis.

作者信息

Buhl L, Muirhead D E, Prentis P F

机构信息

Department of Pathology, College of Medicine, Sultan Qaboos University, Al Khod, Muscat, Sultanate of Oman.

出版信息

Ultrastruct Pathol. 1993 Mar-Apr;17(2):169-83. doi: 10.3109/01913129309084037.

Abstract

A kidney biopsy specimen with pronounced hemosiderosis from a patient with beta-thalassemia major was studied by light and electron microscopy, including X-ray microanalysis. Ferritin was absorbed from the glomerular ultrafiltrate through the parietal epithelial cells and the tubular epithelial cells and from the blood through the endothelial cells. It was transported in siderosomes into the surrounding basal lamina, where electron-dense deposits of hemosiderin were found in the outer part of the lamina densa and the reticular lamina. Fibrosis was seen as a reaction to the iron followed by severe atrophy of affected structures.

摘要

对一名重型β地中海贫血患者的肾活检标本进行了研究,该标本有明显的含铁血黄素沉着,采用了光镜和电镜检查,包括X射线微量分析。铁蛋白通过壁层上皮细胞和肾小管上皮细胞从肾小球超滤液中吸收,并通过内皮细胞从血液中吸收。它被转运到含铁小体中进入周围的基膜,在基膜致密层的外部和网状层中发现了含铁血黄素的电子致密沉积物。纤维化被视为对铁的反应,随后是受影响结构的严重萎缩。

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