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β地中海贫血充分输血后的影像学表现。

The radiographic appearances following adequate transfusion in beta-thalassaemia.

作者信息

Scutellari P N, Orzincolo C, Franceschini F, Bagni B

机构信息

Institute of Radiology, University of Ferrara School of Medicine, Italy.

出版信息

Skeletal Radiol. 1989;17(8):545-50. doi: 10.1007/BF02569398.

Abstract

The main lesions of the skull and hand, observed in a group of hypertransfused beta-thalassaemic patients, are compared with a control group of low-transfused patients. Bony abnormalities reflect the relationship between proliferating bone marrow and bone cortex, and hypertransfusion therapy will prevent development of lesions only if established early in life. If this is done, the diploë in the skull may become normal, overgrowth of facial bone is moderate, pneumatisation of the paranasal sinuses is not completely prevented, and the "hair-brush" pattern may disappear completely. A normal appearance of the hand in adequately treated patients differentiates between prepubertal patients and adults.

摘要

对一组接受大量输血的β地中海贫血患者所观察到的颅骨和手部主要病变,与一组接受少量输血的患者对照组进行了比较。骨质异常反映了增生的骨髓与骨皮质之间的关系,且只有在生命早期开始进行大量输血治疗,才能预防病变的发展。如果做到这一点,颅骨的板障可能恢复正常,面骨过度生长程度适中,鼻窦气化不能完全得到预防,且“毛刷”样表现可能完全消失。经过充分治疗的患者手部外观正常,这可区分青春期前患者和成年人。

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