Department of Pediatrics, University of Alberta, 4-584 Edmonton Clinic Health Academy, 11405 - 87 Ave, Edmonton, AB, T6G 2R7, Canada.
Women's and Children's Health Research Institute, Alberta, Canada.
Osteoporos Int. 2022 Nov;33(11):2397-2408. doi: 10.1007/s00198-022-06455-2. Epub 2022 Jul 29.
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.
To evaluate bone morbidity and the response to intravenous (IV) bisphosphonate therapy in children with SCD.
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).
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.
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.
评估儿童镰状细胞病 (SCD) 患者的骨骼发病率及静脉用双膦酸盐治疗的反应。
我们对加拿大三家儿科三级护理中心 2003 年至 2019 年的患者病历进行了回顾性分析。对影像学（包括 X 线片、磁共振成像、计算机断层扫描）检查结果进行评估，以明确有无发生股骨头坏死 (AVN)、骨梗死和骨肌炎。我们为骨骼疼痛患者提供静脉用双膦酸盐治疗。通过双能 X 线吸收法 (DXA) 评估骨密度。
46 名儿童（20 名女性，43%）存在骨骼发病率，平均年龄为 11.8 岁（标准差 3.9 岁），包括股骨（17/46，37%）和肱骨（8/46，17%）头部的 AVN、因终板梗死导致的 H 形椎体畸形（35/46，76%）和非椎体骨骼梗死（15/46，32%）。5 名儿童（5/26，19%）在磁共振成像上发现 AVN 或骨梗死区域存在肌炎。23 名儿童（8 名女性）接受了静脉用双膦酸盐治疗。所有患者均报告称骨骼疼痛显著或完全缓解。未报告有因静脉用双膦酸盐治疗而导致镰状细胞溶血性危象、疼痛危象或中风的情况。
儿童 SCD 患者有发生广泛且早期骨骼发病率的可能。在本研究中，静脉用双膦酸盐治疗可有效缓解骨骼疼痛，且不会引发镰状细胞并发症。
