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血清硬骨素水平与儿童血友病性关节病的骨密度。

Serum Sclerostin Level and Bone Mineral Density in Pediatric Hemophilic Arthropathy.

机构信息

Department of Physical Medicine, Rheumatology and Rehabilitation, Ain Shams University Hospitals, Faculty of Medicine, Ain Shams University, Cairo, Egypt.

Department of Pediatrics, Ain Shams University Hospitals, Faculty of Medicine, Ain Shams University, Cairo, Egypt.

出版信息

Indian J Pediatr. 2019 Jun;86(6):515-519. doi: 10.1007/s12098-019-02855-1. Epub 2019 Jan 22.

Abstract

OBJECTIVE

To assess serum sclerostin levels in relation to severity of arthropathy and bone mineral density (BMD) in children with hemophilic arthropathy.

METHODS

This cross-sectional study included 40 male children suffering from Hemophilia A, and 10 matched healthy controls. Assessment of factor VIII deficiency degree, frequency of bleeding, type of treatment, body mass index (BMI), disease severity using the Hemophilia Joint Health Score (HJHS) and lumbar spine (LS) Z score for bone mineral density (BMD) using dual-energy X-ray absorbiometry was done. Serum sclerostin levels were measured for all patients and controls.

RESULTS

Significant difference of serum sclerostin levels between the patient and control groups with Mean ± SD (0.09 ± 0.07 ng/ml) and (0.04 ± 0.01 ng/ml) (P value = 0.028) respectively was found. Significant positive correlations between serum sclerostin levels and the patients' age, and HJHS (P value <0.05) were found, while it had negative correlation with DEXA Z score, not reaching a significant value. LS-BMD-Z score levels ranged from (-4.5 to 1.2), with 15 patients with low BMD Z score (less than -2) representing 37.5% of total patients.

CONCLUSIONS

Serum sclerostin levels are elevated in hemophilic children denoting bone metabolism affection and correlates with increased age, and HJHS. Increased levels of serum sclerostin may identify hemophilic patients at high risk for developing osteoporosis.

摘要

目的

评估血友病性关节炎患儿血清硬骨素水平与关节炎严重程度和骨密度(BMD)的关系。

方法

本横断面研究纳入了 40 名男性血友病 A 患儿和 10 名匹配的健康对照。评估因子 VIII 缺乏程度、出血频率、治疗类型、体重指数(BMI)、使用血友病关节健康评分(HJHS)评估疾病严重程度,使用双能 X 线吸收仪评估腰椎(LS)BMD Z 评分。对所有患者和对照组进行血清硬骨素水平检测。

结果

患者组和对照组的血清硬骨素水平分别为 Mean ± SD(0.09 ± 0.07 ng/ml)和(0.04 ± 0.01 ng/ml)(P 值=0.028),差异有统计学意义。血清硬骨素水平与患者年龄和 HJHS 呈显著正相关(P 值<0.05),与 DEXA Z 评分呈负相关,但无统计学意义。LS-BMD-Z 评分范围为(-4.5 至 1.2),15 名患者 BMD Z 评分较低(低于-2),占总患者的 37.5%。

结论

血友病患儿血清硬骨素水平升高,提示骨代谢异常,与年龄增加和 HJHS 相关。血清硬骨素水平升高可能有助于识别易发生骨质疏松症的血友病患儿。

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