驱铜联合活性维生素D3治疗对Wilson's病患者骨骼及代谢的影响

目的观察Wilson's病患者骨骼X线摄片表现,及驱铜治疗联合活性维生素D3补充治疗对骨代谢的影响。方法对35例入选Wilson's病患者入院时进行骨骼X线摄片,并分别于治疗前及二巯基丙磺酸钠驱铜辅以活性维生素D3治疗8疗程后,放射免疫法测定骨代谢相关激素:PTH、CT、及血清BGP水平;用生化法测定血钙、血磷,尿钙、尿磷等。并用单光子吸收法(SPA)测定尺桡骨中远端1/3处平均骨密度值。结果Wilson's病患者手腕部X线检测异常率达60%。治疗后,血PTH、血钙均降低,骨密度(BMD)值升高,较治疗前差异有统计学意义(P<0·05);血磷、尿钙、磷及血清CT、BGP水平较治疗前差异无显著性。结论Wilson's病患者常并发骨质疏松或骨质软化等代谢性骨病,驱铜治疗联合活性维生素D3补充治疗可更好改善骨骼代谢,更快改善其骨密度。

The effect of decopper therapy accompanied by supply active vitamin D3 on skeleton and its metabolism in Wilson's disease

ObjectiveTo observe X-ray photographs of skeleton in patients with Wilson's disease, and the effect of decopper therapy accompanied by supply active vitamin D_3 on skeletal metabolism.MethodsTook X-ray photographs of skeleton in 35 cases of patients with Wilson's disease upon admission. Before and after 8 courses of Decopper treatment accompanied by supply active vitamin D_3, we determined the serumal hormones of PTH, CT and serumal BGP which are relative to skeletal metabolism by RIA. The levels of serumal calcium, phosphate and uric calcium, phosphate were determined with biochemical methods. One third point from distal end of ulna and radius was measured with single photon absorptiometry (SPA) for bone mineral density (BMD).ResultsThe abnormal ratio of roentgen rays photogram reaches 60% within phalanges, metacarpus, and carpal bones of the Wilson's patients. After treatment, the levels of serumal calcium, PTH remarkably degraded and the value of BMD significantly increased (P<0.05). The other indexes such as serumal BGP, CT, serumal phosphate and uric calcium, phosphate had no significant change.ConclusionsMetabolic bone diseases such as osteoporosis or osteomalacia exist in patients with Wilson's disease. Decopper therapy accompanied by supply active vitamin D_3 could preferably improve skeletal metabolism and quickly raise the value of BMD.