[一名患有进行性假性风湿性关节发育不良的 15 岁患者的双侧全髋关节置换术。病例报告和文献综述]。

G Garabano, A Jaime, M I Alonso, C A Pesciallo
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引用次数: 0

摘要

进行性假性类风湿发育不良(PPD)是一种发病率极低的常染色体隐性遗传病。该病的特点是多关节受累,从很小的时候就会出现关节炎和进行性畸形,严重影响患者的生活质量。只有通过基因检测才能确诊,目前还没有特效药物治疗。在髋关节受累的情况下,一种治疗方法是关节置换术。在本病例报告中,我们介绍了一名继发于 PPD 的双侧髋关节病的 15 岁男孩,他分两个阶段接受了双侧全髋关节置换术。我们重点介绍了这种罕见疾病的特点、术中发现、功能结果以及对生活质量的影响。
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[Bilateral total hip arthroplasty in a 15-year-old patient with progressive pseudorheumatoid dysplasia. Case report and literature review].

Progressive pseudorheumatoid dysplasia (PPD) is an autosomal recessive hereditary disease of very low prevalence. It is characterized by the affection of multiple joints, generating arthrosis and progressive deformities from a very young age, which significantly affect the quality of life of patients. Its diagnosis is only confirmed by genetic testing, and no specific pharmacological treatment is still available. In the case of hip involvement, one treatment option is arthroplasty. In this case report, we present a 15-year-old boy with bilateral coxarthrosis secondary to PPD who underwent bilateral total hip arthroplasty in two stages. We highlight the characteristics of this rare entity, the intraoperative findings, the functional outcomes, and the impact on quality of life.

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