Prevalence of fractures among the Thalassemia syndromes in North America
Prevalence of fractures among the Thalassemia syndromes in North America
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DOI：
10.1016/j.bone.2005.10.001
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发表时间：
2006-04-01
期刊：
影响因子：
4.1
通讯作者：
Giardina, PJ
中科院分区：
文献类型：
--
作者：
Vogiatzi, MG;Macklin, EA;Giardina, PJ
从历史上看，在优化输血和螯合方案之前，骨折被认为是地中海贫血患者的常见问题。本研究的目的是确定骨折的患病率在当代样本的北美地中海贫血患者。使用北美地中海贫血临床研究网络（TCRN）数据库登记系统收集了702例诊断为常见α和β地中海贫血的患者的历史数据，包括重型地中海贫血（TM）、中间型地中海贫血（TI）、E/β、纯合子α地中海贫血（AT）、血红蛋白H病（HbH）和HbH伴恒定弹簧（HbH/CS），这些患者同意进行病历审查。DXA骨密度（BMD）测量结果可用于回顾一个亚组的患者（n = 312）。所有地中海贫血综合征的总骨折患病率为12.1%，平均分布于女性（11.5%）和男性（12.7%）。TM（16.6%）和TI（12.2%）的骨折发生率高于E/Beta（7.4%）和alpha（2.3%）。患病率随着年龄的增长而增加（0 - 10岁2.5%，11 - 19岁7.4%，> 20岁23.2%），并随着性激素替代疗法（SHRT）的使用而增加（P <0.01）。平均而言，有骨折史的低阴离子患者的BMD Z和T评分为0.85 SD（平均Z/T评分为-2.78 vs.-1.93，95%CI差异为-0.49至-1.22 SD）。P = 0.02）。存在其他内分泌疾病（即甲状腺功能减退症，甲状旁腺功能减退症和糖尿病），人体测量参数，心脏病或丙型肝炎是不显着的独立predictors of fractures.These数据表明，骨折仍然是一个常见的并发症之间的老年患者TM和TI β-地中海贫血。然而，与20世纪60年代至70年代发表的报告相比，骨折患病率有所改善。此外，地中海贫血儿童与一般人群相比，骨折率较低。(c)2005年爱思唯尔公司All rights reserved.
Historically, fractures are cited as a frequent problem in patients with Thalassemia prior to optimization of transfusion and chelation regimens. The aim of this study was to determine the prevalence of fractures in a contemporary sample of North American patients with Thalassemia. The North American Thalassemia Clinical Research Network (TCRN) database registry was used to gather historical data on 702 patients with common alpha and beta-Thalassemia diagnoses including Thalassemia Major (TM), Intermedia (TI), E/Beta, homozygous alpha Thalassemia (AT), Hemoglobin H disease (HbH) and HbH with Constant Spring (HbH/CS), who consented to a medical record chart review. Bone mineral density (BMD) measurements by DXA were available for review in a subgroup of patients (n = 312).The overall fracture prevalence among all Thalassemia syndromes was 12.1%, equally distributed between females (11.5%) and males (12.7%). Fractures occurred more frequently in TM (16.6%) and TI (12.2%) compared to E/Beta (7.4%) and alpha (2.3%). Prevalence increased with age (2.5% ages 0-10 years, 7.4% ages 11-19 years, 23.2% ages > 20 years) and with use of sex hormone replacement therapy (SHRT) (P < 0.01). On average, BMD Z and T scores were 0.85 SD lower anion patients with a history of fractures (mean Z/T score -2.78 vs. -1.93, 95% Cl for the difference -0.49 to -1.22 SD. P = 0.02). Presence of other endocrinopathies (i.e. hypothyroidism, hypoparathyroidism and diabetes mellitus), anthropometric parameters, heart disease or hepatitis C were not significant independent predictors of fractures.These data indicate that fractures remain a frequent complication among the aging patients with both TM and TI beta-Thalassemia. However, the fracture prevalence has improved compared to published reports from the 1960s to 1970s. In addition, children with Thalassemia appeal-to have low fracture rates compared to the general population. (c) 2005 Elsevier Inc. All rights reserved.