威尔逊病中的骨质疏松症:一项大型队列研究,强调年龄、性别和骨骼症状是临床监测的关键危险因素。

IF 3.5 2区 医学 Q2 GENETICS & HEREDITY
Ling Zhu, Bin Song, Yun Xu, Yu-Long Zhu, Lei Hua, Na Nian, Long Zhang, Quan Sun, Ben-Chun Xue, Yin Xu, Yong-Sheng Han
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引用次数: 0

摘要

背景:威尔逊氏病(WD)是一种罕见的影响铜代谢的疾病,其特征是多器官系统损伤,包括肝、脑和眼睛。WD患者存在骨密度(BMD)降低的风险。只有少数研究调查了WD和BMD之间的关系,并且数据存在差异。因此,我们调查了WD患者的骨密度状况,分析了影响骨量变化的危险因素。方法:本回顾性队列研究选择2018年1月至2024年8月在中国合肥一家神经医院住院的426例WD患者作为研究对象。入组患者分为骨质疏松组(13例)、骨质减少组(99例)和正常骨量组(314例)。计算骨质疏松和骨质减少的患病率,采用多元有序logistic回归分析骨质疏松和骨质减少的危险因素。结果:WD患者骨质疏松和骨质减少的患病率分别为3.1%和23.2%。多因素有序logistic回归分析显示,年龄、男性、病程中出现骨骼症状是WD患者骨质疏松、骨质减少的独立危险因素,比值比(OR)(95%可信区间[CI])分别为1.103(1.074 ~ 1.134)、2.292(1.216 ~ 4.320)、2.675(1.395 ~ 5.131)。结论:年龄较大、男性、病程中出现骨骼症状的WD患者易发生骨质疏松和骨质减少改变。临床应加强对此类患者的骨密度监测和早期干预。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Osteoporosis in Wilson's disease: A large cohort study highlighting age, sex and skeletal symptoms as key risk factors for clinical surveillance.

Background: Wilson's disease (WD) is a rare disorder affecting copper metabolism that is characterized by multiple organ system damage, including the liver, brain, and eyes. Patients with WD are at risk for decreased bone mineral density (BMD). Only a few studies have investigated the relationship between WD and BMD, and there are discrepancies in the data. Therefore, we investigated the BMD status of patients with WD and analyzed the risk factors affecting the bone mass change.

Methods: This retrospective cohort study selected 426 patients with WD who were admitted to a neurological hospital in Hefei, China, from January 2018 to August 2024 as study subjects. The enrolled patients were divided into the osteoporosis group (13 patients), osteopenia group (99 patients), and normal bone mass group (314 patients). The rates of prevalence of osteoporosis and osteopenia were calculated, and the risk factors of osteoporosis and osteopenia were analyzed by multivariate ordered logistic regression.

Results: The prevalence of osteoporosis and osteopenia in patients with WD was 3.1% and 23.2%, respectively. Multivariate ordered logistic regression analysis demonstrated that age, male sex, and the presence of skeletal symptoms during the course of the disease were independent risk factors for osteoporosis and osteopenia in patients with WD, with odds ratio (OR) (95% confidence interval [CI]) values of 1.103 (1.074-1.134), 2.292 (1.216-4.320), and 2.675 (1.395-5.131), respectively.

Conclusions: Patients with WD with older age, male sex, and skeletal symptoms during the course of the disease are prone to osteoporosis and osteopenia changes. BMD monitoring and early intervention of such patients should be strengthened clinically.

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来源期刊
Orphanet Journal of Rare Diseases
Orphanet Journal of Rare Diseases 医学-医学:研究与实验
CiteScore
6.30
自引率
8.10%
发文量
418
审稿时长
4-8 weeks
期刊介绍: Orphanet Journal of Rare Diseases is an open access, peer-reviewed journal that encompasses all aspects of rare diseases and orphan drugs. The journal publishes high-quality reviews on specific rare diseases. In addition, the journal may consider articles on clinical trial outcome reports, either positive or negative, and articles on public health issues in the field of rare diseases and orphan drugs. The journal does not accept case reports.
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