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血友病患者的骨质疏松症:来自中等收入国家的单中心结果。

Osteoporosis in Patients With Hemophilia: Single-Center Results From a Middle-Income Country.

机构信息

1 Division of Endocrinology, Metabolism and Diabetes, Department of Internal Medicine, Cerrahpasa Faculty of Medicine, Istanbul University-Cerrahpasa, Istanbul, Turkey.

2 Division of Hematology, Department of Internal Medicine, Faculty of Medicine, Istanbul Medipol University, Istanbul, Turkey.

出版信息

Clin Appl Thromb Hemost. 2019 Jan-Dec;25:1076029619861689. doi: 10.1177/1076029619861689.

Abstract

Increased number of patients with hemophilia have been identified to have osteoporosis at early ages. Low bone mineral density in the setting of hemophilia has been associated with decreased mobility, sedentary life style, on demand treatment or delayed prophylaxis, low body weight and viral infections. The aim of this study was to investigate the impact of hemophilia on bone health of adult patients living in a middle income country. A total of 61 adult patients with hemophilia who were followed at the Hematology Department of Cerrahpaşa Medical Faculty, Istanbul University-Cerrahpaşa were consecutively included in this study. Bone health of the patients was assessed using the bone mineral density (BMD) and vitamin D levels. Z and t scores are used for evaluation of BMD in patients with hemophilia aged < 50 and ≥ 50 years, respectively. Information on treatment and co-morbidities including viral diseases were obtained from the medical files of the recruited patients. Bone mineral density was found normal in 30, and low in 29 patients. Vitamin D levels were below 20 ng/ml in 46 patients. No significant relationship was found between the severity of hemophilia and bone density. Vitamin D levels were significantly lower in patients who had a history of joint intervention. Neither annual bleeding rate nor the treatment modality (on demand versus prophylaxis) were associated with the bone mineral density and vitamin D levels. Annual factor consumption was higher in patients whose bone mineral densities was low both in femoral and lumbar regions. The results of this study depicting the situation of adult hemophilia population from a middle income country show that bone mineral density and vitamin D levels were decreased in a considerable amount of patients at early ages.

摘要

越来越多的血友病患者在年轻时就被发现患有骨质疏松症。血友病患者的骨矿物质密度低与活动能力下降、久坐的生活方式、按需治疗或延迟预防、低体重和病毒感染有关。本研究旨在探讨中低收入国家成年血友病患者的骨骼健康状况。共有 61 名在伊斯坦布尔大学-切拉帕萨医学院血液科接受治疗的成年血友病患者连续纳入本研究。通过骨矿物质密度(BMD)和维生素 D 水平评估患者的骨骼健康状况。年龄<50 岁和≥50 岁的血友病患者分别使用 Z 评分和 t 评分评估 BMD。从纳入患者的病历中获取关于治疗和合并症(包括病毒疾病)的信息。结果发现,30 名患者的骨矿物质密度正常,29 名患者的骨矿物质密度低。46 名患者的维生素 D 水平低于 20ng/ml。未发现血友病严重程度与骨密度之间存在显著关系。有过关节介入史的患者维生素 D 水平明显较低。年出血率和治疗方式(按需治疗与预防治疗)均与骨矿物质密度和维生素 D 水平无关。在股骨和腰椎区域骨矿物质密度均较低的患者中,每年因子消耗更高。这项来自中低收入国家的成年血友病人群的研究结果表明,相当数量的患者在年轻时就出现了骨矿物质密度和维生素 D 水平降低的情况。

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