特立帕肽治疗严重骨质疏松、甲状旁腺功能减退和地中海贫血1例

A. Graziani, M. Cannito, M. Putti, V. Camozzi
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摘要

β-地中海贫血(TM)是β-地中海贫血的一种形式。TM并发症包括骨质疏松症,其发生率在TM患者中从13.6%到50%不等。tcm相关骨质疏松症的整体病因机制尚不清楚。治疗TM患者骨质疏松的主要方法是治疗TM及其并发症,并使用抗吸收药物,如双膦酸盐(bp)作为一线治疗药物。在这篇文章中,我们提出了一例45岁的女性TM和严重骨质疏松症,多处骨折,尽管假设bp多年。bp在TM患者中的抗骨折疗效和安全性尚不明确。数据显示,依地膦酸和唑来膦酸应被视为治疗TM相关骨质疏松症的一线药物。关于特立帕肽(Teriparatide, TP)在TM患者中的应用,仅有少数病例报道。值得注意的是,在TP治疗结束时,其益处迅速丧失。最后,关于romosozumab,由于存在胰岛素治疗的糖尿病(DM)和tm相关心肌病,我们的患者存在显著的心血管风险,建议我们避免使用该药物。本病例报告表明,治疗骨质疏松症的患者TM仍然是一个开放的问题。TM患者经常出现多种合并症,这限制了骨质疏松症的治疗。此外,这些合并症往往是骨质疏松症不可避免的危险因素。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Teriparatide in a patient with severe osteoporosis, hypoparathyroidism and thalassemia major
β-Thalassemia Major (TM) is a form of beta-thalassemias. TM complications include, among others, osteoporosis, whose frequency, among TM patients, varies from 13.6% to 50%. The overall etiological mechanisms of TM-related osteoporosis remain unclarified. The primary approach to osteoporosis in patients with TM is the management of TM and its complications and the use of antiresorptive agents, such as Bisphosphonates (BPs), as the first line-drug of treatment. In this article, we present the case of 45 years old-woman with TM and severe osteoporosis, with multiple fractures, albeit the assumption of BPs for many years. The anti-fracture efficacy and safety of BPs are not well-established in TM patients. Data suggest that etidronate and zoledronic acid should be considered as first-line agents in the management of TM- associated osteoporosis. Regarding Teriparatide (TP), there are only a few case reports published about its use in TM patients. It is also noticed that, at the dismission of TP therapy, its benefits are rapidly lost. Finally, regarding romosozumab, our patient presents a significant cardiovascular risk due to the presence of insulin-treated Diabetes Mellitus (DM) and TM-related cardiomyopathy, suggesting we avoid this drug. This case report shows that the therapy of osteoporosis in patients with TM remains an open problem. TM patients often present multiple comorbidities which create limitations to osteoporosis’s treatment. Moreover, these comoboridites are often unavoidable risk factors for osteoporosis.
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