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[智利骨佩吉特病:15例报告]

[Paget disease of bone in Chile: report of 15 cases].

作者信息

González Gilberto, Brusco Francisca, Arteaga Lugenio, Rodríguez José, Jacobelli Sergio, Massardo Loreto, Ortiz Cristián, Contreras Oscar

机构信息

Departamentos de Endocrinología, Facultad de Medicina, Pontificia Universidad Católica de Chile.

出版信息

Rev Med Chil. 2003 May;131(5):491-7.

Abstract

BACKGROUND

Paget disease of bone (PD) is a localized disorder of bone remodeling, which leads to bone fragility and deformity. In Chile PD is uncommon.

AIM

To study clinical and demographic characteristics of patients with PD seen in the Clinical Hospital of the Catholic University.

PATIENTS AND METHODS

Patients with typical radiological and clinical features of PD referred to our institution during the last decade were included in this review.

RESULTS

We obtained data from 15 patients with PD (ten males, eight Chilean, six European and one Asian), eleven of them were diagnosed during the last 3 years. The mean age at diagnosis was 68.7 +/- 11.1 years old. No one had first degree relatives with PD. Bone pain was the main complaint in 13 patients and elevated total alkaline phosphatases in the other two. The average duration of the symptoms prior to diagnosis was 38.8 months. Eight patients had monostotic lesions; the most commonly involved sites were the pelvis, spine and femur. Radiological evaluation disclosed sclerotic changes in all patients as well as bone deformity and osteoarthritis in eight patients. Total alkaline phosphatases were elevated in 14 cases (mean: 4 times over the upper normal limit).

CONCLUSIONS

When compared to series of the Northern hemisphere, PD in Chile is characterized by an older age at diagnosis, a higher frequency of symptomatic presentation, advanced radiological involvement and greater proportion of complications. PD should be suspected in every patient, Chilean or foreigner, with bone pain or elevated alkaline phosphatases.

摘要

背景

骨佩吉特病(PD)是一种局部性骨重塑紊乱疾病,可导致骨质脆弱和畸形。在智利,PD并不常见。

目的

研究在天主教大学临床医院就诊的PD患者的临床和人口统计学特征。

患者与方法

本综述纳入了过去十年间转诊至我院、具有典型PD放射学和临床特征的患者。

结果

我们获取了15例PD患者的数据(10例男性,8例智利人,6例欧洲人,1例亚洲人),其中11例在过去3年被诊断出。诊断时的平均年龄为68.7±11.1岁。无人有患PD的一级亲属。13例患者的主要症状为骨痛,另外2例患者总碱性磷酸酶升高。诊断前症状的平均持续时间为38.8个月。8例患者为单骨病变;最常受累部位为骨盆、脊柱和股骨。放射学评估显示所有患者均有硬化改变,8例患者有骨畸形和骨关节炎。14例患者总碱性磷酸酶升高(平均:超过正常上限4倍)。

结论

与北半球的系列研究相比,智利的PD具有诊断年龄较大、症状性表现频率较高、放射学受累程度较高以及并发症比例较大的特点。对于每一位有骨痛或碱性磷酸酶升高的患者,无论其为智利人还是外国人,都应怀疑患有PD。

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