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佩吉特病在印度存在吗?:21例患者系列研究。

Does Paget's disease exist in India?: A series of 21 patients.

作者信息

Bhadada S, Bhansali A, Unnikrishnan A G, Khadgawat R, Singh S K, Mithal A, Saikia U N

机构信息

Department of Endocrinology, PGIMER, Chandigarh.

出版信息

J Assoc Physicians India. 2006 Jul;54:530-4.

Abstract

OBJECTIVE

Paget's disease of bone has been described as a few case reports from India. The aim of the present study is to document the existence of Paget's disease (PD) in India.

MATERIAL AND METHODS

We describe demography, clinical manifestations, biochemical and radiological profile and the treatment outcome of 21 patients of PD.

RESULTS

Mean (+/-SD) age of these patients at presentation was 49.2 +/- 17.6 years and the male to female ratio was 2.5:1. Common clinical manifestations included backache, headache and bone pains. Others were fracture, joint pain, deafness, gait ataxia, visual impairment and difficulty in biting. Two patients presented with hydrocephalus and one had recurrent paraparesis. Fifteen (71.4%) patients had polyostotic and six (28.6%) had monoostotic Paget's disease. More commonly involved bones were skull and spine (61.9%) followed by pelvis (38.1%), femur (33.3%), tibia (9%) and ulna (9%). Mean (+/-SD) serum alkaline phosphatase at diagnosis was 1514 +/- 1168 IU/L and nine months after treatment with bisphosphonates decreased to 454 +/- 406 IU/ L(P<0.03).

CONCLUSION

This illustrates that Paget's disease does exist in India and a high index of suspicion is required to clinch the diagnosis.

摘要

目的

印度已有关于骨佩吉特病的少数病例报告。本研究的目的是记录印度骨佩吉特病(PD)的存在情况。

材料与方法

我们描述了21例骨佩吉特病患者的人口统计学特征、临床表现、生化和放射学特征以及治疗结果。

结果

这些患者就诊时的平均(±标准差)年龄为49.2±17.6岁,男女比例为2.5:1。常见临床表现包括背痛、头痛和骨痛。其他症状有骨折、关节疼痛、耳聋、步态共济失调、视力障碍和咬合困难。两名患者出现脑积水,一名患者反复出现下肢轻瘫。15例(71.4%)患者为多骨型骨佩吉特病,6例(28.6%)为单骨型骨佩吉特病。较常受累的骨骼是颅骨和脊柱(61.9%),其次是骨盆(38.1%)、股骨(33.3%)、胫骨(9%)和尺骨(9%)。诊断时血清碱性磷酸酶的平均(±标准差)水平为1514±1168 IU/L,使用双膦酸盐治疗9个月后降至454±406 IU/L(P<0.03)。

结论

这表明印度确实存在骨佩吉特病,确诊需要高度的怀疑指数。

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