血友病关节病的骨健康评估:一项来自印度西孟加拉邦加尔各答的单中心研究

P. Mandal, Malini Garg, Prantar Chakrabarti, A. Bhowmik, Debasis Gantait, T. Dolai
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The number of joints affected by haemophilic arthropathy was recorded. Hemophilia Joint Health Score (HJHS) and Pettersson score were calculated for each patient. Levels of serum calcium, phosphorus, vitamin D3 and b-ALP were assayed in all cases. Results A total of 320 PWH were included; the majority (85%; 272/320) had severe haemophilia, 13.44% (43/320) moderate haemophilia and 1.56% (5/320) mild haemophilia. With increasing age, the number of joints involved increased significantly (r=0.2250, p<0.05). When adjusted for age, b-ALP was higher than normal for the majority of PWH (88.75%). Increased number of joints involved and severity of disease had a positive correlation with higher-than-normal b-ALP (adjusted for age) (r=0.2112, p=0.0001). A significant positive correlation was seen between Pettersson score and HJHS score (r=0.1126, p=0.04). There was no significant correlation between number of joints involved and serum vitamin D3 level across the whole cohort. (p<0.05). 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摘要

血友病与低骨密度(BMD)患病率增加有关,而低骨密度反过来又可能加重血友病关节病。双能x射线吸收测定法(DEXA)是评估骨密度的黄金标准,但在印度并没有广泛使用。骨转换的标志物,如骨特异性碱性磷酸酶(b-ALP)反映成骨细胞转换,可能是低骨密度的替代品。目的探讨血友病(PWH)患者血清维生素D3和b-ALP水平与关节病变程度的相关性,评价血友病患者的骨健康状况。方法在横断面研究中,纳入了所有严重程度的血友病A和B患者,关节病变累及≥3个关节。记录受血友病关节病影响的关节数量。计算每位患者血友病关节健康评分(HJHS)和Pettersson评分。测定所有病例血清钙、磷、维生素D3和b-ALP水平。结果共纳入PWH 320例;大多数人(85%;重度血友病患者占13.44%(43/320),轻度血友病患者占1.56%(5/320)。随着年龄的增加,受累关节数显著增加(r=0.2250, p<0.05)。经年龄调整后,大多数PWH患者的b-ALP高于正常水平(88.75%)。累及关节数量的增加和疾病的严重程度与高于正常水平的b-ALP呈正相关(经年龄调整)(r=0.2112, p=0.0001)。Pettersson评分与HJHS评分呈显著正相关(r=0.1126, p=0.04)。在整个队列中,关节数量与血清维生素D3水平之间没有显著相关性。(p < 0.05)。结论重度PWH和血友病性关节病患者b-ALP高于正常水平,反映了骨翻转增加和骨密度降低。因此,b-ALP可能是一种有用的标志物,可以帮助评估PWH患者的骨骼健康状况,特别是在DEXA扫描受限的情况下。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Bone health assessment in haemophilic arthropathy: A single centre study from Kolkata, West Bengal, India
Abstract Background Haemophilia has been associated with increased prevalence of low bone mineral density (BMD) which in turn may aggravate haemophilic arthropathy. Dual-energy X-ray absorptiometry (DEXA) is the gold standard for assessing BMD but is not widely available across India. Markers of bone turnover like bone-specific alkaline phosphatase (b-ALP) reflect osteoblastic turnover and may be surrogate to low BMD. Aim To evaluate how bone health in people with haemophilia (PWH) can be assessed by serum vitamin D3 and b-ALP level, correlated with the degree of arthropathy. Methods In this cross-sectional study, people with haemophilia A and B of all severities with arthropathy involving ≥3 joints were included. The number of joints affected by haemophilic arthropathy was recorded. Hemophilia Joint Health Score (HJHS) and Pettersson score were calculated for each patient. Levels of serum calcium, phosphorus, vitamin D3 and b-ALP were assayed in all cases. Results A total of 320 PWH were included; the majority (85%; 272/320) had severe haemophilia, 13.44% (43/320) moderate haemophilia and 1.56% (5/320) mild haemophilia. With increasing age, the number of joints involved increased significantly (r=0.2250, p<0.05). When adjusted for age, b-ALP was higher than normal for the majority of PWH (88.75%). Increased number of joints involved and severity of disease had a positive correlation with higher-than-normal b-ALP (adjusted for age) (r=0.2112, p=0.0001). A significant positive correlation was seen between Pettersson score and HJHS score (r=0.1126, p=0.04). There was no significant correlation between number of joints involved and serum vitamin D3 level across the whole cohort. (p<0.05). Conclusion PWH with severe disease and haemophilic arthropathy have higher than normal b-ALP, which in turn reflects increased bone turn over and low BMD. Hence, b-ALP may be a useful marker to help assess bone health in PWH, particularly in settings where access to DEXA scans is constrained.
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