Bone mass and metabolism in thalassemic children and adolescents treated with different iron-chelating drugs
Bone mass and metabolism in thalassemic children and adolescents treated with different iron-chelating drugs
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DOI：
10.1007/s00774-003-0449-z
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发表时间：
2004-01-01
影响因子：
3.3
通讯作者：
Isaia, GC
中科院分区：
文献类型：
--
作者：
Di Stefano, M;Chiabotto, P;Isaia, GC
我们评估了22例纯合子青春期前β地中海贫血患者接受去铁胺治疗后的骨密度（BMD）和骨转换。10例患者在整个研究期间接受去铁胺治疗，而12例患者停用去铁胺，然后接受去铁酮（L1）治疗。在基线和随访1年和3年后检查腰椎和股骨BMD和骨代谢指标。所有患者在基线时均处于青春期前，在3年随访期间均进入青春期。基线时，平均腰椎Z评分值为-2.048 SD +/- 0.75; 13例儿童的Z评分小于-2 SD，6例在-1和-2 SD范围内，仅3例受试者在0和-1 SD范围内。3年后，在腰椎（+8.466%/年）和股骨水平（颈部平均+3.46%/年，转子间区域平均+5.83%/年）观察到BMD显著增加（P < 0.0001），在去铁胺治疗的患者和L1治疗的患者之间没有任何显著差异。平均Z评分SD值在1年时增加至-1.957 +/- 0.975（与基线无显著差异），在3年随访时增加至-1.864 +/- 1.221（与基线相比P < 0.05）;还观察到骨转换增加。这些研究结果表明，低BMD，β地中海贫血的标志，显着改善青春期开始时，这种增加涉及不同的骨骼部位，无论用不同的铁螯合药物的药物治疗。
We evaluated bone mineral density (BMD) and bone turnover in 22 homozygous prepubertal beta-thalassemic patients treated with desferrioxamine. Ten patients underwent treatment with desferrioxamine for the whole study period, while 12 patients stopped desferrioxamine and were then treated with deferiprone (L1). Lumbar and femoral BMD and bone metabolism markers were examined at baseline and after 1 and 3 years of follow up. All patients were prepubertal at baseline and they all became pubertal over the 3 years of follow up. At baseline, the mean lumbar Z score value was -2.048 SD +/- 0.75; the Z score was less than -2 SD in 13 children, within -1 and -2 SD in 6, and within 0 and -1 SD in only 3 subjects. A significant BMD increase (P < 0.0001) was observed at both the lumbar (+8.466%/year) and the femoral level (average of +3.46%/year at neck and +5.83%/year at the intertrochanteric region) after 3 years, without any significant difference being shown between patients treated with desferrioxamine and those treated with L1. The mean Z score SD values increased to -1.957 +/- 0.975 at 1 year (not significantly different from baseline) and to -1.864 +/- 1.221 at 3 year follow up (P < 0.05 vs baseline); an increase in bone turnover was also observed. These findings show that low BMD, a hallmark of beta-thalassemia, improves significantly when puberty begins; this increase involves different skeletal sites, regardless of pharmacological treatment with different iron-chelating drugs.