Scutellari P N, Orzincolo C, Franceschini F, Bagni B, Atti G
Istituto di Radiologia, Arcispedale S. Anna, Ferrara.
Radiol Med. 1988 Nov;76(5):399-404.
The main abnormalities of the hand and skull in a group of patients who underwent hypertransfusion regimen were reviewed and compared to the lesions observed in a control group of low-transfused patients. The results are reported, and a possible correlation with transfusion regimen is discussed: since bone lesions are explained on the basis of relationship between proliferating bone marrow and bone cortex, hypertransfusion regimen will prevent the development of abnormalities, only if established early in life. For this reason, skeletal lesions in Cooley's anaemia have changed their "classical" radiographic patterns. In the skull, the diploic space may become normal, and the overgrowth of facial bones moderate; the pneumatization of paranasal sinuses may not be completely impeded by the swelling of the jaws, the "hair-brush" pattern may disappear completely. The normalized picture of the hand is and remains a typical differential sign of prepuberal and adult patients.
回顾了一组接受高输血方案治疗的患者手部和颅骨的主要异常情况,并与一组低输血患者对照组中观察到的病变进行了比较。报告了结果,并讨论了与输血方案可能存在的相关性:由于骨病变是根据增殖性骨髓与骨皮质之间的关系来解释的,高输血方案只有在生命早期确立才能预防异常情况的发生。因此,库利贫血的骨骼病变改变了其“经典”的放射学模式。在颅骨方面,板障间隙可能恢复正常,面骨过度生长情况减轻;鼻窦的气化可能不会完全被颌骨肿胀所阻碍,“毛刷”样表现可能完全消失。手部的正常化表现一直是青春期前和成年患者的典型鉴别体征。