Carmina E, Di Fede G, Napoli N, Renda G, Vitale G, Lo Pinto C, Bruno D, Malizia R, Rini G B
Department of Clinical Medicine and Emerging Pathologies, University of Palermo, Via del Vespro 147, 90127 Palermo, Italy.
Calcif Tissue Int. 2004 Jan;74(1):68-71. doi: 10.1007/s00223-002-1044-3. Epub 2003 Oct 6.
We studied bone mass and metabolism in 30 adult women (age 28.5 +/- 1.3) with thalassemia major (TM) and evaluated whether prolonged hormone replacement therapy (HRT) was able to optimize bone accrual. TM patients had reduced bone mass, increased bone turnover and lower serum gonadotropin and estradiol levels compared with 10 normal women of similar age. A significant correlation was found between bone mass and sex hormone levels. Six TM patients with normal ovarian function had normal bone turnover markers and modestly low bone mass (lumbar spine -1.29 +/- 0.31; femoral neck -0.60+/-0.21; Z-score). The other 24 TM women were hypogonadic and had significantly lower bone mass for age (lumbar spine -2.35 +/- 0.2, femoral neck -1.83 +/- 0.2) and increased bone turnover relative to eugonadal women. Of the hypogonadal patients, 13 had taken HRT since age 15 +/- 1 years, but their bone mass and turnover markers were not different than untreated hypogonadal patients. In conclusion, while hypogonadism negatively affects bone mass acquisition in adult TM women, HRT at the standard replacement doses is not sufficient to secure optimal bone accrual.
我们研究了30名成年重型地中海贫血（TM）女性（年龄28.5±1.3岁）的骨量和骨代谢，并评估了长期激素替代疗法（HRT）是否能够优化骨量积累。与10名年龄相仿的正常女性相比，TM患者的骨量减少、骨转换增加，血清促性腺激素和雌二醇水平降低。骨量与性激素水平之间存在显著相关性。6名卵巢功能正常的TM患者骨转换标志物正常，骨量略低（腰椎-1.29±0.31；股骨颈-0.60±0.21；Z评分）。其他24名TM女性性腺功能减退，相对于性腺功能正常的女性，她们的年龄相关骨量显著降低（腰椎-2.35±0.2，股骨颈-1.83±0.2），骨转换增加。在性腺功能减退的患者中，13名自15±1岁起接受HRT，但她们的骨量和转换标志物与未接受治疗的性腺功能减退患者并无差异。总之，性腺功能减退对成年TM女性的骨量获取有负面影响，但标准替代剂量的HRT不足以确保最佳骨量积累。
