Bone disease in thalassemia: a frequent and still unresolved problem.
Bone disease in thalassemia: a frequent and still unresolved problem.
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thalalsyaine骨骼疾病：一个频繁且仍未解决的问题。
DOI：
10.1359/jbmr.080505
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发表时间：
2009-03
期刊：
影响因子：
--
通讯作者：
Thalassemia Clinical Research Network
中科院分区：
文献类型：
--
作者：
Vogiatzi MG;Macklin EA;Fung EB;Cheung AM;Vichinsky E;Olivieri N;Kirby M;Kwiatkowski JL;Cunningham M;Holm IA;Lane J;Schneider R;Fleisher M;Grady RW;Peterson CC;Giardina PJ;Thalassemia Clinical Research Network
患有重型β地中海贫血的成年人通常具有低BMD、骨折和骨痛。本研究的目的是确定所有地中海贫血综合征在儿童、青少年和成年期的低BMD、骨折和骨痛的患病率，BMD与骨折和骨痛的关系，以及地中海贫血骨病的病因。地中海贫血临床研究网络中的所有地中海贫血综合征患者，年龄≥6岁，既往无影响骨量或需要类固醇的疾病，均参与研究。我们通过DXA测量脊柱和股骨BMD以及全身BMC，并通过形态测量X线吸收法（MXA）评估椎体异常。通过访谈和病历审查、体格检查以及血液和尿液采集进行病史检查。361例受试者，49%为男性，平均年龄23.2岁（范围6.1-75岁），进行了研究。脊柱和股骨BMD Z评分<-2的参与者分别为46%和25%。年龄大、体重低、性腺功能减退和骨转换增加是低骨量的独立预测因素，与地中海贫血综合征无关。峰值骨量不理想。36%的患者有骨折史，34%的患者报告骨痛。BMD与骨折呈负相关，但与骨痛无关。9%的参与者通过MXA发现几个椎骨的高度均匀降低，这与6岁之前使用铁螯合剂去铁胺有关。在地中海贫血患者中，低BMD和骨折频繁发生，且与特定综合征无关。峰值骨量不理想。低BMD与性腺功能减退、骨转换增加和骨折风险增加有关。
Adults with β thalassemia major frequently have low BMD, fractures, and bone pain. The purpose of this study was to determine the prevalence of low BMD, fractures, and bone pain in all thalassemia syndromes in childhood, adolescence, and adulthood, associations of BMD with fractures and bone pain, and etiology of bone disease in thalassemia. Patients of all thalassemia syndromes in the Thalassemia Clinical Research Network, ≥6 yr of age, with no preexisting medical condition affecting bone mass or requiring steroids, participated. We measured spine and femur BMD and whole body BMC by DXA and assessed vertebral abnormalities by morphometric X-ray absorptiometry (MXA). Medical history by interview and review of medical records, physical examinations, and blood and urine collections were performed. Three hundred sixty-one subjects, 49% male, with a mean age of 23.2 yr (range, 6.1–75 yr), were studied. Spine and femur BMD Z-scores < −2 occurred in 46% and 25% of participants, respectively. Greater age, lower weight, hypogonadism, and increased bone turnover were strong independent predictors of low bone mass regardless of thalassemia syndrome. Peak bone mass was suboptimal. Thirty-six percent of patients had a history of fractures, and 34% reported bone pain. BMD was negatively associated with fractures but not with bone pain. Nine percent of participants had uniformly decreased height of several vertebrae by MXA, which was associated with the use of iron chelator deferoxamine before 6 yr of age. In patients with thalassemia, low BMD and fractures occur frequently and independently of the particular syndrome. Peak bone mass is suboptimal. Low BMD is associated with hypogonadism, increased bone turnover, and an increased risk for fractures.
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影响因子：
4.2
作者：
Molyvda-Athanasopoulou, E;Sioundas, A;Vainas, I
通讯作者：
Vainas, I
影响因子：
3.3
作者：
Di Stefano, M;Chiabotto, P;Isaia, GC
通讯作者：
Isaia, GC
影响因子：
5.1
作者：
EHLERS, KH;GIARDINA, PJ;HILGARTNER, MW
通讯作者：
HILGARTNER, MW
DOI：
10.1016/s0020-1383(97)00144-7
发表时间：
1997-11-01
影响因子：
2.5
作者：
Johansen, A;Evans, RJ;Woodhouse, KW
通讯作者：
Woodhouse, KW
影响因子：
4
作者：
Jones, IE;Williams, SM;Goulding, A
通讯作者：
Goulding, A