Pediatric Hematology-Oncology Department, Kerman Medical Sciences University, Kerman, Iran.
Arch Iran Med. 2012 Feb;15(2):82-4.
Types A and B hemophilia are coagulation disorders associated with many complications. Osteoporosis is a skeletal condition characterized by the decreased density of normally mineralized bone. This study aims to determine the relationship between osteoporosis and hemophilic arthropathy in severe hemophilia patients over the age of 20 years in Kerman, Iran.
We performed a cross-sectional study of bone density among 40 male patients with severe hemophilia. Lumbar spine and femoral bone mineral density (BMDs) were measured using a Dual Energy X-ray Absorptiometry (DEXA) scan. The T-scores for BMDs were computed and values from -2.5 to -1 were considered as osteopenia. Those less than -2.5 were considered to be osteoporotic.
About 42% of patients had normal BMD, 50% were osteopenic, and the rest were osteoporotic. The mean BMI, number of arthropathic joints, and the numbers of joint bleeding during the previous year were significantly higher in osteoporotic patients than osteopenic and normal groups (P = 0.05, = 0.003 and = 0.011, respectively). The mean for factor replacement, the number of joint bleeding episodes in the past year, and the number of arthropathic joints were significant independent predictors of both spinal and femoral BMD. Their odds ratios (OR) were 1.29 (factor replacement), 1.17 (numbers of joint bleeding episodes), and 1.73 (number of arthropathic joints), which were significant (P < 0.05).
Our results suggest that men with severe hemophilia have reduced BMD. Patients at risk are those with signs of hemophilic arthropathy. Because osteoporosis may complicate the future treatment of patients with hemophilia, screening of patients with hemophilic arthropathy for reduction of BMD and preventive therapies is highly recommended.
A型和 B 型血友病是与许多并发症相关的凝血障碍。骨质疏松症是一种骨骼疾病,其特征是正常矿化骨密度降低。本研究旨在确定伊朗克尔曼 20 岁以上严重血友病患者骨质疏松症与血友病性关节炎之间的关系。
我们对 40 名严重血友病男性患者进行了骨密度的横断面研究。使用双能 X 射线吸收仪(DEXA)扫描测量腰椎和股骨骨矿物质密度(BMD)。计算 BMD 的 T 评分,将 -2.5 至-1 的值视为骨量减少。小于-2.5 的值被认为是骨质疏松症。
约 42%的患者 BMD 正常,50%为骨量减少,其余为骨质疏松症。骨质疏松症患者的平均 BMI、关节病关节数以及前一年关节出血次数均明显高于骨量减少组和正常组(P=0.05,=0.003 和=0.011)。因子替代的平均值、前一年关节出血次数以及关节病关节数是脊柱和股骨 BMD 的显著独立预测因素。它们的比值比(OR)分别为 1.29(因子替代)、1.17(关节出血次数)和 1.73(关节病关节数),均有统计学意义(P<0.05)。
我们的结果表明,严重血友病男性患者的 BMD 降低。有患病风险的是那些有血友病性关节炎迹象的患者。由于骨质疏松症可能会使血友病患者的未来治疗复杂化,因此强烈建议对有血友病性关节炎的患者进行 BMD 降低的筛查和预防性治疗。