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8例畸形性骨炎的临床诊治分析
The clinical diagnosis and treatment in 8 cases with Paget`s disease of bone
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DOI:
作者:
鲍春华, 章振林
Bao Chun-hua, Zhang Zhen-lin
作者单位:
上海交通大学附属第六人民医院骨质疏松和骨病专科 骨代谢病和遗传研究室
Metabolic Bone Disease and Genetic Research Unit, Department of Osteoporosis and Bone Diseases, Shanghai Jiao Tong University Affiliated Sixth People’s Hospital, Shanghai, P.R. China
关键词:
畸形性骨炎;诊断;双膦酸盐,治疗
Paget’s disease of bone; Diagnosis; Bisphosphonates; Treatment
摘要:
目的 通过对8例畸形性骨炎的临床诊治的分析,旨在提高我们对该病的认识。 方法 确诊畸形性骨炎患者8例,对其临床表现、X线特征、骨转换生化指标及用双膦酸盐的治疗进行介绍。 结果 1、男性5例,女性3例,平均年龄(60.0±12.2)岁 (46-76岁)。2、平均病程 (5.1±5.0)年(1-15年)。3、临床症状表现为受累骨骼的疼痛8例, 其中2例伴局部皮温的增高。4、病变累及部位:单骨性损害和多处骨骼受累分别为3例和4例,1例未能明确,最常见于骨盆、股骨和脊柱;其次为头颅、胫骨、腓骨和肱骨。5、X线影像学显示受损骨的骨小梁结构粗大和紊乱、皮质显著增厚、髓腔缩小和骨骼变形等典型Paget`s骨损害。6、骨扫描显示受累骨骼的核素异常浓聚。7、8例患者均行血碱性磷酸酶(ALP)测定,为93-1366U/L(中位数326U/L),其中1例血ALP为正常水平。8、6例给予双膦酸盐治疗畸形性骨炎,3月后疼痛缓解,活动功能改善,血ALP水平下降显著,疗效明显。结论 尽管畸形性骨炎在我国罕见,常误诊,但X线检查和骨核素扫描等影像学表现结合骨转换生化指标的显著改变是诊断的重要依据,静脉用双膦酸盐治疗疗效显著。
Objective To improve our knowledge of diagnosis and treatment of Paget’s disease of bone, X-ray features, the biochemical marker of bone turnover and the treatment of the Paget’s disease of bone were analyzed. Methods Eight patients with Paget’s disease of bone were diagnosed and introduce the clinical manifestation, X-ray features, change of the biochemical marker of bone turnover and the results of treatment. Results 1. 5 males and 3 females, average aged (60.0±12.2)years (range 46-76 years)。 2. The average course of disease was (5.1±5.0) years(range 1-15 years). 3. It was clinically manifested by the pain, deformity of the bone involved. Two patients were found the temperature increase of local surface of bone. 4. Bone lesions of 3 patients were limited in single site, while 4 patients had multiple bone lesions. The commonest sites were pelvis, spine and femurs; the secondly were skull, tibia, fibula, humeral bone, radial bone. 5. X-ray film showed the classic features of Paget`s impaired bone. 6. Bone scan showed increased uptake of 99mTC-Bisphosphonate in involved bone. 7. Serum alkaline phosphatase (ALP) was detected in all patients when they visit before therapy, from 93u/L to 1366u/L(median 326u/L). Only one case had normal serum ALP level. 8. Seven patients were treated by diphosphonates. Diphosphonates reduced the Pagetic bone pain, significantly decreased serum ALP levels, and were effective on Paget’s disease of bone. Conclusion Paget’s disease of bone is very rare in China. The important diagnostic evidence is the remarkable change of biochemical marker of bone turnover and the typical radiological features. Bisphosphonates in vein is effective on Paget’s disease of bone.
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