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肩部戈勒姆-斯托特病:临床、病理及治疗考量

Gorham-Stout Disease of the Shoulder: Clinical, Pathologic and Therapeutic Considerations.

作者信息

Fares Mohamad Y, Fares Jawad, Fares Youssef, Abboud Joseph A

机构信息

The Rothman Institute, Department of Orthopaedic Surgery, Thomas Jefferson University Hospital, Philadelphia, PA, USA.

Faculty of Medicine, American University of Beirut, Beirut, Lebanon.

出版信息

Arch Bone Jt Surg. 2020 Jan;8(1):58-69. doi: 10.22038/abjs.2019.37156.1990.

DOI:10.22038/abjs.2019.37156.1990
PMID:32090147
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7007711/
Abstract

BACKGROUND

Gorham-Stout disease (GSD) is a rare skeletal disorder characterized by massive osteolysis of a bony area in the body. When it hits the shoulder, the patient is faced with a debilitating limitation in terms of motion, stability and quality of life. GSD etiology and pathology are unknown and, as a result, therapeutic modalities remain unclear. The aim of this paper is to explore and offer medical insight into the possible etiologies, pathologies and therapeutic modalities of GSD in the shoulder.

METHODS

We explored PubMed/Medline for GSD cases in the shoulder. The search involved all articles published from database inception until February 1, 2019. Only articles published in English were included. Demographics and clinical information extrapolated from the reported cases were analyzed to deduce patterns and infer conclusions.

RESULTS

Only 32 studies met our criteria, with a total of 37 cases (n=37). Males predominated in 21 cases (57%). Twelve cases (32%) were younger than 18 years, and 18 cases (49%) were aged between 18 and 65 years. Shoulder pain was the predominant reporting symptom. The humerus was the most common shoulder site affected (54%), followed by the scapula (35%) and the clavicle (30%). Almost half of the cases affected the right shoulder (51%), the left shoulder was affected in 16 cases (43%). Conservative treatment was opted in 17 cases (46%), while surgery was performed in 13 cases (35%). Good outcomes were reported in 28 cases (76%), while death occurred twice (5%).

CONCLUSION

Understanding the demographics and clinical characteristics of GSD in the shoulder region will help in formulating better therapeutic interventions and preventive health policies.

摘要

背景

戈勒姆-斯托特病(GSD)是一种罕见的骨骼疾病,其特征是身体某一骨区域出现大量骨质溶解。当它累及肩部时,患者在活动、稳定性和生活质量方面会面临严重限制。GSD的病因和病理尚不清楚,因此治疗方式也不明确。本文旨在探讨并提供有关肩部GSD可能的病因、病理和治疗方式的医学见解。

方法

我们在PubMed/Medline中搜索肩部GSD病例。搜索涵盖了从数据库建立到2019年2月1日发表的所有文章。仅纳入英文发表的文章。对从报告病例中推断出的人口统计学和临床信息进行分析,以推断模式并得出结论。

结果

只有32项研究符合我们的标准,共37例(n = 37)。男性占21例(57%)。12例(32%)年龄小于18岁,18例(49%)年龄在18至65岁之间。肩部疼痛是主要报告症状。肱骨是肩部最常受累的部位(54%),其次是肩胛骨(35%)和锁骨(30%)。几乎一半的病例累及右肩(51%),16例(43%)累及左肩。17例(46%)选择保守治疗,13例(35%)进行了手术。28例(76%)报告预后良好,2例(5%)死亡。

结论

了解肩部区域GSD的人口统计学和临床特征将有助于制定更好的治疗干预措施和预防性健康政策。

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