2nd Propedeutic Department of Internal Medicine, Sarantaporou 10, Greece.
Thromb Haemost. 2013 Aug;110(2):257-63. doi: 10.1160/TH13-01-0030. Epub 2013 Jun 6.
Haemophilia A and B have been associated with increased prevalence of low bone mineral density (BMD). However, no study has so far evaluated the effects of anti-osteoporotic therapy on BMD in haemophilia.The primary endpoint of this prospective study was to estimate the effect of 12-month therapy of oral ibandronate 150 mg/month on BMD in patients with haemophilia A and B. Secondary endpoint was its effect on turnover markers (BTM) of bone resorption [serum C-terminal telopeptide of type 1 collagen (sCTX), tartrate-resistant acid phosphatase band 5b] and bone formation (osteocalcin and bone-specific alkaline phosphatase. Ten adult patients with T-score < -2.5 SD or Z-score < -2 and/or increased risk of fracture according to FRAX model were included. All received 1,000 mg/day calcium carbonate with 800 IU/d cholecalciferol. Males with haemophilia A (n=7) or B (n=3) (mean age 43.5 ± 13.5 years) were studied. Ibandronate resulted in an increase in lumbar BMD (from 0.886 ± 0.169 to 0.927 ± 0.176 g/cm2, 4.7%, p=0.004). No change in BMD of total hip (from 0.717 ± 0.128 to 0.729 ± 0.153 g/cm2, p=0.963) or femoral neck (0.741 ± 0.135 to 0.761 ± 0.146 g/cm2, p=0.952) was noticed. Ibandronate led to a decrease in sCTX (from 0.520 ± 0.243 to 0.347 ± 0.230 ng/ml, -29.9%, p=0.042). No change was observed in other BTM. Ibandronate was generally well-tolerated. In conclusion, ibandronate significantly improved BMD in lumbar spine and reduced bone resorption in adults with haemophilia at increased risk of fracture. Its effect on hip BMD and bone formation markers was not significant.
血友病 A 和 B 与低骨密度(BMD)的患病率增加有关。然而,迄今为止,尚无研究评估抗骨质疏松治疗对血友病患者 BMD 的影响。这项前瞻性研究的主要终点是估计每月口服伊班膦酸钠 150mg 治疗 12 个月对血友病 A 和 B 患者 BMD 的影响。次要终点是其对骨吸收(血清 1 型胶原 C 端肽(sCTX)、抗酒石酸酸性磷酸酶 5b)和骨形成(骨钙素和骨特异性碱性磷酸酶)的转换标志物的影响。纳入了 10 名 T 评分<-2.5SD 或 Z 评分<-2 且根据 FRAX 模型有骨折风险增加的成年患者。所有患者均接受 1000mg/天碳酸钙和 800IU/天胆钙化醇。7 名男性血友病 A(n=7)或 B(n=3)(平均年龄 43.5±13.5 岁)患者接受了研究。伊班膦酸钠使腰椎 BMD 增加(从 0.886±0.169 增加至 0.927±0.176g/cm2,增加 4.7%,p=0.004)。全髋关节(从 0.717±0.128 增加至 0.729±0.153g/cm2,p=0.963)或股骨颈(0.741±0.135 增加至 0.761±0.146g/cm2,p=0.952)的 BMD 无变化。伊班膦酸钠导致 sCTX 降低(从 0.520±0.243 降低至 0.347±0.230ng/ml,降低 29.9%,p=0.042)。其他 BTM 无变化。伊班膦酸钠通常耐受性良好。总之,伊班膦酸钠可显著改善骨折风险增加的成年血友病患者的腰椎 BMD,并降低骨吸收。其对髋关节 BMD 和骨形成标志物的影响不显著。