Research Article

地中海贫血主要诱导的骨质疏松症中的骨代谢标志物:来自横断面观察研究的结果

卷 19, 期 5, 2019

页: [335 - 341] 页: 7

弟呕挨: 10.2174/1566524019666190314114447

价格: $65

摘要

背景:重型地中海贫血(TM)患者最终面临许多新的健康状况,包括内分泌病和低骨密度,通常在老年人群中观察到。目的:本研究的目的是评估TM患者骨重建的生物标志物,并与骨质疏松症和健康人群进行比较,以探讨新的治疗途径。 方法:64名TM患者(32名男性和32名女性)参与了该研究。使用腰椎和股骨颈的双能X射线吸收测定法(DXA)和骨重建标志物评估患者,包括核因子κ-κB受体激活剂(RANKL),骨保护素(OPG),C-末端。端肽(CTX)和硬化蛋白。将结果与12名骨质疏松症绝经后妇女和12名骨密度正常的妇女进行比较。 结果:骨代谢的生化标志物的统计分析显示,仅RANKL和OPG / RANKL的三组之间存在总体显着差异(p = 0.049和p = 0.009)。与骨质疏松症组相比,TM患者的RANKL较高且OPG / RANKL较低。 结论:患有TM的患者患一般人群的骨质疏松症的可能性较高。然而,TM和骨质疏松症患者的破骨细胞活性的一些标志物不同,表明在抗骨质疏松症治疗方面可能存在差异。三组之间在CTX和硬化蛋白水平方面缺乏显着差异,这可能表明目前骨质疏松治疗对TM患者的潜在功效。

关键词: 骨质疏松症,重型地中海贫血,硬化蛋白,RANKL,OPG,内分泌病,骨代谢。

[1]
Weatherall DJ. In:The Molecular Basis of Blood Diseases. Stamatoyannopoulos, G,Nienhuis, AW,Majerus, PW,Varmus, H, Eds. Philadelphia, PA: WB Saunders. 1994; p. 815.
[2]
Dresner Pollack R, Rachmilewitz E, Blumenfeld A, Idelson M, Goldfarb AW. Bone mineral metabolism in adults with beta-thalassaemia major and intermedia. Br J Haematol 2000; 111: 902-7.
[3]
Sharma R, Seth A, Chandra J, et al. Endocrinopathies in adolescents with thalassaemia major receiving oral iron chelation therapy. Paediatr Int Child Health 2016; 36: 22-7.
[4]
Molyvda-Athanasopoulou E, Sioundas A, Karatzas N, et al. Bone mineral density of patients with thalassemia major: four-year follow-up. Calcif Tissue Int 1999; 64: 481-4.
[5]
Voskaridou E, Stoupa E, Antoniadou L, et al. Osteoporosis and osteosclerosis in sickle cell/beta-thalassemia: the role of the RANKL/osteoprotegerin axis. Haematologica 2006; 91: 813-6.
[6]
Spatz JM, Wein MN, Gooi JH, et al. The Wnt inhibitor sclerostin is up-regulated by mechanical unloading in osteocytes in vitro. J Biol Chem 2015; 290: 16744-58.
[7]
McClung MR, Grauer A, Boonen S, et al. Romosozumab in postmenopausal women with low bone mineral density. N Engl J Med 2014; 370: 412-20.
[8]
van Dinther M, Zhang J, Weidauer SE, et al. Anti-Sclerostin antibody inhibits internalization of Sclerostin and Sclerostin-mediated antagonism of Wnt/LRP6 signaling. PLoS One 2013; 8: e62295.
[9]
Cosman F, Crittenden DB, Adachi JD, et al. Romosozumab treatment in postmenopausal women with osteoporosis. N Engl J Med 2016; 375: 1532-43.
[10]
Voskaridou E, Anagnostopoulos A, Konstantopoulos K, et al. Zoledronic acid for the treatment of osteoporosis in patients with beta-thalassemia: results from a single-center, randomized, placebo-controlled trial. Haematologica 2006; 91: 1193-202.
[11]
Morabito N, Gaudio A, Lasco A, et al. Osteoprotegerin and RANKL in the pathogenesis of thalassemia-induced osteoporosis: new pieces of the puzzle. J Bone Miner Res 2004; 19: 722-7.
[12]
Meena MC, Hemal A, Satija M, Arora SK, Bano S. Comparison of bone mineral density in thalassemia major patients with healthy controls. Adv Hematol 2015; 2015: 648349.
[13]
Voskaridou E, Kyrtsonis MC, Terpos E, et al. Bone resorption is increased in young adults with thalassaemia major. Br J Haematol 2001; 112: 36-41.
[14]
Gratwick GM, Bullough PG, Bohne WH, Markenson AL, Peterson CM. Thalassemic osteoarthropathy. Ann Intern Med 1978; 88: 494-501.
[15]
De Sanctis V, Pinamonti A, Di Palma A, et al. Growth and development in thalassaemia major patients with severe bone lesions due to desferrioxamine. Eur J Pediatr 1996; 155: 368-72.
[16]
Lasco A, Morabito N, Gaudio A, et al. Osteoporosis and beta-thalassemia major: role of the IGF-I/IGFBP-III axis. J Endocrinol Invest 2002; 25: 338-44.
[17]
Li X, Zhang Y, Kang H, et al. Sclerostin binds to LRP5/6 and antagonizes canonical Wnt signaling. J Biol Chem 2005; 280: 19883-7.
[18]
Wijenayaka AR, Kogawa M, Lim HP, et al. Sclerostin stimulates osteocyte support of osteoclast activity by a RANKL-dependent pathway. PLoS One 2011; 6: e25900.
[19]
Voskaridou E, Ntanasis-Stathopoulos I, Papaefstathiou A, et al. Denosumab in transfusion-dependent thalassemia osteoporosis: a randomized, placebo-controlled, double-blind phase 2b clinical trial. Blood Adv 2018; 2: 2837-47.
[20]
Gaudio A, Morabito N, Catalano A, et al. Pathogenesis of thalassemia major-associated osteoporosis: Review of the literature and our experience. J Clin Res Pediatr Endocrinol 2018.
[21]
Geusens P. New insights into treatment of osteoporosis in postmenopausal women. RMD Open 2015; 1: e000051.
[22]
MacNabb C, Patton D, Hayes JS. Sclerostin antibody therapy for the treatment of osteoporosis: Clinical prospects and challenges. J Osteoporos 2016; 2016: 6217286.
[23]
Voskaridou E, Christoulas D, Plata E, et al. High circulating sclerostin is present in patients with thalassemia-associated osteoporosis and correlates with bone mineral density. Horm Metab Res 2012; 44: 909-13.

Rights & Permissions Print Cite
© 2024 Bentham Science Publishers | Privacy Policy