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突尼斯骨佩吉特病:69例患者的研究

Paget's Disease of Bone in Tunisia: A Study of 69 Patients.

作者信息

Maatallah Kaouther, Rahmouni Safa, Miladi Saoussen, Rouached Leila, Ferjeni Hanen, Fazaa Alia, Laatar Ahmed, Kaffel Dhia, Hamdi Wafa

机构信息

Department of Rheumatology, Kassab Orthopedics Institute, Ksar Saïd, Research Unit UR 17SP04, Faculty of Medicine of Tunis, Tunisia.

Department of Rheumatology, Mongi Slim Hospital, La Marsa, Faculty of Medicine of Tunis, University Tunis el Manar, Tunis, Tunisia.

出版信息

Indian J Endocrinol Metab. 2020 Sep-Oct;24(5):422-427. doi: 10.4103/ijem.IJEM_239_20. Epub 2020 Nov 9.

Abstract

CONTEXT

Paget's disease of bone is a common bone disease with a striking variation in its incidence and characteristics in different parts of the world. It is uncommonly reported in African patients.

AIMS

Given the lack of studies describing the characteristics of patients with Paget's disease of bone in North Africa, we aimed to describe demographic, clinical, biochemical, and imaging characteristics, as well as treatment outcomes of Tunisian patients with Paget's disease of bone.

SUBJECTS AND METHODS

This bicentric and retrospective study included patients with Paget's disease of bone. Clinical, laboratory, radiological profile, and response to treatment were analyzed.

RESULTS

Sixty-nine patients were identified. The mean age was 64.9 ± 11.6 years and 52.2% were women. One patient reported a positive family history. Seven patients were asymptomatic. Bone pain was the most common presenting symptom. Eight patients had a history of malignancy. In three patients, Paget's disease of bone was diagnosed as part of a metastatic workup. Monostotic disease was found in half of the cases. The most commonly involved sites were pelvis (43.5%), femur (21.7%), and spine (21.7%). The mean serum alkaline phosphatase level at presentation was 591 U/L (68-8380). Two patients received salmon calcitonin (2.8%) and 47 patients (68.1%) received bisphosphonates. After a mean follow-up of 55 months (2-240 months), bone pain improved in 43.1% of patients and the serum alkaline phosphate levels had normalized in 22 of them (43.1%). During follow-up, there was no malignant transformation.

CONCLUSIONS

In this series of Tunisian patients, Paget's disease of bone had a female predominance and was usually monostotic. The clinical and radiological presentations were similar to the European series.

摘要

背景

骨佩吉特病是一种常见的骨病,在世界不同地区其发病率和特征存在显著差异。在非洲患者中鲜有报道。

目的

鉴于缺乏描述北非骨佩吉特病患者特征的研究,我们旨在描述突尼斯骨佩吉特病患者的人口统计学、临床、生化和影像学特征以及治疗结果。

对象与方法

这项双中心回顾性研究纳入了骨佩吉特病患者。分析了临床、实验室、放射学特征及治疗反应。

结果

共确定69例患者。平均年龄为64.9±11.6岁,女性占52.2%。1例患者有阳性家族史。7例患者无症状。骨痛是最常见的首发症状。8例患者有恶性肿瘤病史。3例患者的骨佩吉特病是在转移性检查中被诊断出来的。半数病例为单骨型疾病。最常累及的部位是骨盆(43.5%)、股骨(21.7%)和脊柱(21.7%)。就诊时血清碱性磷酸酶平均水平为591 U/L(68 - 8380)。2例患者接受了鲑鱼降钙素治疗(2.8%),47例患者(68.1%)接受了双膦酸盐治疗。平均随访55个月(2 - 240个月)后,43.1%的患者骨痛有所改善,其中22例患者(43.1%)的血清碱性磷酸酶水平恢复正常。随访期间未发生恶性转化。

结论

在这组突尼斯患者中,骨佩吉特病以女性居多,通常为单骨型。临床和放射学表现与欧洲系列相似。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c996/7810052/6d94cccb7dc1/IJEM-24-422-g001.jpg

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