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去铁胺诱发的重型地中海贫血患者骨发育异常

Deferoxamine-induced bone dysplasia in patients with thalassemia major.

作者信息

Brill P W, Winchester P, Giardina P J, Cunningham-Rundles S

机构信息

Department of Radiology, New York Hospital-Cornell Medical Center, NY 10021.

出版信息

AJR Am J Roentgenol. 1991 Mar;156(3):561-5. doi: 10.2214/ajr.156.3.1899759.

DOI:10.2214/ajr.156.3.1899759
PMID:1899759
Abstract

Metaphyseal irregularity and abnormal vertebral bodies resembling a bone dysplasia were seen in two of five children with thalassemia major who were begun on a regimen of hypertransfusion and chelation with deferoxamine before the age of 3 years. Similar changes were not seen in 22 other children in whom chelation was started after the age of 3. Whether the dysplastic bone growth was related to drug dose or age of onset of chelation could not be determined, as deferoxamine dosages differed in the two groups. Findings on radiographs included flattening of the thoracic and lumbar vertebral bodies, circumferential metaphyseal osseous defects, sharp zones of provisional calcification, and widened growth plates. Healing was noted in one of the patients after the dose of deferoxamine was decreased. Zinc levels in both affected patients did not differ from those in the 25 other chelated patients.

摘要

在5名重型地中海贫血患儿中,有2名在3岁前开始接受强化输血和去铁胺螯合治疗,其干骺端不规则及椎体异常类似骨发育异常。在3岁后开始螯合治疗的其他22名儿童中未见到类似变化。由于两组去铁胺剂量不同,因此无法确定发育异常的骨骼生长是否与药物剂量或螯合治疗开始的年龄有关。X线片表现包括胸腰椎椎体扁平、干骺端周缘骨质缺损、临时钙化带清晰、生长板增宽。1例患者在去铁胺剂量减少后可见好转。2名受累患者的锌水平与其他25名接受螯合治疗的患者无差异。

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