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地中海贫血的影像学特征。

Imaging features of thalassemia.

作者信息

Tunaci M, Tunaci A, Engin G, Ozkorkmaz B, Dinçol G, Acunaş G, Acunaş B

机构信息

Department of Radiology, Faculty of Medicine, Istanbul University, Istanbul, Turkey.

出版信息

Eur Radiol. 1999;9(9):1804-9. doi: 10.1007/s003300050926.

Abstract

Thalassemia is a kind of chronic, inherited, microcytic anemia characterized by defective hemoglobin synthesis and ineffective erythropoiesis. In all thalassemias clinical features that result from anemia, transfusional, and absorptive iron overload are similar but vary in severity. The radiographic features of beta-thalassemia are due in large part to marrow hyperplasia. Markedly expanded marrow space lead to various skeletal manifestations including spine, skull, facial bones, and ribs. Extramedullary hematopoiesis (ExmH), hemosiderosis, and cholelithiasis are among the non-skeletal manifestations of thalassemia. The skeletal X-ray findings show characteristics of chronic overactivity of the marrow. In this article both skeletal and non-skeletal manifestations of thalassemia are discussed with an overview of X-ray findings, including MRI and CT findings.

摘要

地中海贫血是一种慢性、遗传性小细胞贫血,其特征为血红蛋白合成缺陷和红细胞生成无效。在所有地中海贫血中,由贫血、输血和铁吸收过载导致的临床特征相似,但严重程度有所不同。β地中海贫血的影像学特征在很大程度上归因于骨髓增生。骨髓腔明显扩大导致各种骨骼表现,包括脊柱、颅骨、面骨和肋骨。髓外造血、含铁血黄素沉着症和胆结石是地中海贫血的非骨骼表现。骨骼X线检查结果显示骨髓慢性过度活跃的特征。本文讨论了地中海贫血的骨骼和非骨骼表现,并概述了X线检查结果,包括MRI和CT检查结果。

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