Summary

Children with sickle cell disease (SCD) have the potential for extensive and early-onset bone morbidity. This study reports on the diversity of bone morbidity seen in children with SCD followed at three tertiary centers. IV bisphosphonates were effective for bone pain analgesia and did not trigger sickle cell complications.

Introduction

To evaluate bone morbidity and the response to intravenous (IV) bisphosphonate therapy in children with SCD.

Methods

We conducted a retrospective review of patient records from 2003 to 2019 at three Canadian pediatric tertiary care centers. Radiographs, magnetic resonance images, and computed tomography scans were reviewed for the presence of avascular necrosis (AVN), bone infarcts, and myositis. IV bisphosphonates were offered for bone pain management. Bone mineral density was assessed by dual-energy X-ray absorptiometry (DXA).

Results

Forty-six children (20 girls, 43%) had bone morbidity at a mean age of 11.8 years (SD 3.9) including AVN of the femoral (17/46, 37%) and humeral (8/46, 17%) heads, H-shaped vertebral body deformities due to endplate infarcts (35/46, 76%), and non-vertebral body skeletal infarcts (15/46, 32%). Five children (5/26, 19%) had myositis overlying areas of AVN or bone infarcts visualized on magnetic resonance imaging. Twenty-three children (8/23 girls) received IV bisphosphonate therapy. They all reported significant or complete resolution of bone pain. There were no reports of sickle cell hemolytic crises, pain crises, or stroke attributed to IV bisphosphonate therapy.

Conclusion

Children with SCD have the potential for extensive and early-onset bone morbidity. In this series, IV bisphosphonates were effective for bone pain analgesia and did not trigger sickle cell complications.

中文翻译：

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儿童镰状细胞性骨病和静脉注射双膦酸盐的反应

概括

患有镰状细胞病 (SCD) 的儿童具有广泛和早发性骨病的可能性。本研究报告了在三个三级中心跟踪的 SCD 儿童的骨骼发病率的多样性。IV 双膦酸盐对骨痛镇痛有效，并且不会引发镰状细胞并发症。

介绍

评估 SCD 儿童的骨发病率和对静脉 (IV) 双膦酸盐治疗的反应。

方法

我们对加拿大三个儿科三级护理中心 2003 年至 2019 年的患者记录进行了回顾性审查。对 X 光片、磁共振图像和计算机断层扫描检查是否存在缺血性坏死 (AVN)、骨梗死和肌炎。静脉注射双膦酸盐用于骨痛管理。通过双能 X 射线骨密度仪 (DXA) 评估骨矿物质密度。

结果

46 名儿童（20 名女孩，43%）在平均年龄 11.8 岁（SD 3.9）时患有骨病，包括股骨头（17/46，37%）和肱骨头（8/46，17%）的 AVN，由于终板梗塞（35/46, 76%）和非椎体骨骼梗塞（15/46, 32%）导致的 H 形椎体畸形。五名儿童 (5/26, 19%) 有肌炎覆盖 AVN 或磁共振成像可见的骨梗死区域。23 名儿童（8/23 名女孩）接受了 IV 双膦酸盐治疗。他们都报告了骨痛的显着或完全消退。没有关于静脉注射双膦酸盐治疗导致镰状细胞溶血危象、疼痛危象或中风的报告。

结论

患有 SCD 的儿童具有广泛和早发性骨病的可能性。在该系列中，IV 双膦酸盐对骨痛镇痛有效，并且不会引发镰状细胞并发症。