Kotb Magd A, Fakhry Sherihan, AbdElBaky Hend, Abd El Khalek Mayada Y, Kamal Naglaa M, Alfifi Jaber, Oshi Mohammed A M, Alalyani Saad A, Basanti Christine
Cairo University, Cairo, Egypt.
University of Bisha, Saudi Arabia.
Sage Open Pediatr. 2025 Sep 11;12:30502225251367480. doi: 10.1177/30502225251367480. eCollection 2025 Jan-Dec.
Wilson's disease is a rare inherited disorder causing copper accumulation, which may adversely affect bone health in children. To assess bone mineral density and the prevalence of osteopenia and osteoporosis in pediatric Wilson's disease patients using DEXA. A cross-sectional study was conducted on 15 children with confirmed Wilson's disease. Bone mineral density was assessed using dual-energy X-ray absorptiometry (DEXA), and clinical, biochemical, and treatment-related variables were analyzed. The mean age was 10 ± 3.6 years; 53% were female. DEXA revealed bone disease in 60%: mild osteopenia (13%), moderate (27%), and osteoporosis (20%). Bone disease did not correlate with treatment duration, urinary copper, calcium, phosphorus, or alkaline phosphatase. However, serum albumin positively correlated with bone density ( = .018). Bone disease is frequent and often subclinical in pediatric Wilson's disease. DEXA is a useful tool for early detection. Larger studies are needed to evaluate the effects of treatment and micronutrient status on bone health.
威尔逊氏病是一种罕见的遗传性疾病，会导致铜蓄积，这可能会对儿童的骨骼健康产生不利影响。使用双能X线吸收法（DEXA）评估儿科威尔逊氏病患者的骨密度以及骨质减少和骨质疏松的患病率。对15名确诊为威尔逊氏病的儿童进行了一项横断面研究。使用双能X线吸收法（DEXA）评估骨密度，并分析临床、生化和治疗相关变量。平均年龄为10±3.6岁；53%为女性。DEXA显示60%的患者存在骨病：轻度骨质减少（13%）、中度（27%）和骨质疏松（20%）。骨病与治疗持续时间、尿铜、钙、磷或碱性磷酸酶无关。然而，血清白蛋白与骨密度呈正相关（=0.018）。在儿科威尔逊氏病中，骨病很常见且往往是亚临床的。DEXA是早期检测的有用工具。需要进行更大规模的研究来评估治疗和微量营养素状况对骨骼健康的影响。
