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去铁胺治疗的重型纯合子β地中海贫血患者的脊柱畸形

Spinal deformities in deferoxamine-treated homozygous beta-thalassemia major patients.

作者信息

Hartkamp M J, Babyn P S, Olivieri F

机构信息

Rijs Universiteit Utrecht, The Netherlands.

出版信息

Pediatr Radiol. 1993;23(7):525-8. doi: 10.1007/BF02012139.

Abstract

A new constellation of spinal changes are observed in homozygous beta-thalassemia major (HBT) patients receiving deferoxamine (DF), an iron-chelating drug used in combination with transfusion therapy in certain anemic syndromes. In a retrospective study of 22 HBT patients who were receiving DF therapy, morphological deformities (decreased spinal height, increased thoracic kyphosis, vertebral flattening and elongation anteriorly, and disk calcification) were found in 16 of 22 patients. These changes are believed to be caused by interference with spinal growth-plate development. Investigation of DF-dose correlation supports the conclusion that the spinal changes were DF-induced. Spinal changes observed in DF-treated patients differ both morphologically and pathogenetically from earlier reports of vertebral deformities occurring as a sequel to compensatory marrow hyperplasia in poorly transfused patients.

摘要

在接受去铁胺(DF)治疗的重型纯合子β地中海贫血(HBT)患者中,观察到一组新的脊柱变化。去铁胺是一种铁螯合剂,在某些贫血综合征中与输血疗法联合使用。在一项对22例接受DF治疗的HBT患者的回顾性研究中,22例患者中有16例出现形态学畸形(脊柱高度降低、胸椎后凸增加、椎体前部扁平及伸长、椎间盘钙化)。这些变化被认为是由于对脊柱生长板发育的干扰所致。对DF剂量相关性的研究支持了脊柱变化是由DF引起的这一结论。接受DF治疗的患者中观察到的脊柱变化在形态学和发病机制上均与早期关于输血不足患者代偿性骨髓增生后遗症所致椎体畸形的报道不同。

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