Stefanopoulos Dimitrios, Papaioannou Nikolaos A, Papavassiliou Athanassios G, Mastorakos George, Vryonidou Andromachi, Michou Aikaterini, Dontas Ismene A, Lyritis George, Kassi Eva, Tournis Symeon
Laboratory for Research of the Musculoskeletal System "Th. Garofalidis", KAT Hospital, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece.
Department of Biological Chemistry, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
J Frailty Sarcopenia Falls. 2018 Mar 1;3(1):13-25. doi: 10.22540/JFSF-03-013. eCollection 2018 Mar.
Homozygous beta-thalassemia represents a serious hemoglobinopathy, in which an amazing prolongation in the survival rate of patients has been achieved over recent decades. A result of this otherwise positive evolution is the fact that bone problems have become a major issue in this group of patients. Through an in-depth review of the related literature, the purpose of this study is to present and comment on the totality of the data that have been published to date pertaining to the prevention and treatment of thalassemia bone-disease, focusing on: the contribution of diet and lifestyle, the treatment of hematologic disease and its complications, the management of hypercalciuria, the role of vitamins and minerals and the implementation of anti-osteoporosis medical regimen. In order to comprehensively gather the above information, we mainly reviewed the international literature through the PubMed database, searching for the preventive and therapeutic data that have been published pertaining to thalassemia bone-disease over the last twenty-nine years. There is no doubt that thalassemia bone-disease is a complication of a multi-factorial etiopathology, which does not follow the rules of classical postmenopausal osteoporosis. Bisphosphonates have been the first line of treatment for many years now, with varied and usually satisfactory results. In addition, over the last few years, more data have arisen for the use of denosumab, teriparatide, and other molecules that are in the clinical trial phase, in beta-thalassemia.
纯合子β地中海贫血是一种严重的血红蛋白病，近几十年来患者的生存率有了惊人的延长。这种在其他方面呈积极进展的结果是，骨骼问题已成为这类患者的一个主要问题。通过对相关文献的深入综述，本研究的目的是呈现并评论迄今已发表的有关地中海贫血骨病预防和治疗的全部数据，重点关注：饮食和生活方式的作用、血液系统疾病及其并发症的治疗、高钙尿症的管理、维生素和矿物质的作用以及抗骨质疏松药物治疗方案的实施。为了全面收集上述信息，我们主要通过PubMed数据库检索国际文献，查找过去29年中已发表的有关地中海贫血骨病的预防和治疗数据。毫无疑问，地中海贫血骨病是一种多因素病因的并发症，并不遵循经典绝经后骨质疏松症的规律。多年来双膦酸盐一直是一线治疗药物，效果多样且通常令人满意。此外，在过去几年中，有关地诺单抗、特立帕肽和其他处于临床试验阶段的分子用于β地中海贫血治疗的更多数据也已出现。
