Abstract：
Men with Klinefelter Syndrome develop some degree of seminiferous tubule degeneration, hyalinization, and fibrosis by adulthood. However, the pathophysiology surrounding testicular fibrosis in Klinefelter Syndrome patients remains incompletely understood.
To perform a systematic review of literature studying the mechanisms of fibrosis initiation or propagation in Klinefelter Syndrome testes.
PubMed was searched systematically for articles specific to Klinefelter Syndrome and the process of fibrosis. Articles that did not contain original data or specifically addressed the target material were excluded. Additional references were extracted when pertinent from the reference lists of included studies.
Primary search yielded 139 articles for abstract review, which was narrowed to 16 for full-text review. Following full-text review, eight contained original data and met topic criteria, with one paper added from reference review for a total of nine papers.
The date range for included papers was 1992-2022. The proposed mechanisms of fibrosis mainly were centered around the impact of altered Sertoli cells on germ cells, the hormonal impact on Leydig cells, the inflammation mediated by mast cells, or the fibrous extracellular matrix deposition by peritubular myoid cells. Additionally, discussions of the role of the altered microvasculature and the specific proteins involved in the blood-testis barrier or the seminiferous tubule architecture are reviewed. Recent papers have incorporated advanced sequencing and offer future directions for targeted gene expression analysis. Still, much of the published data consists solely of immunohistological assessment by age range, creating difficulties in extrapolating causality.
The specific initiating factors of fibrosis of the seminiferous tubules and the propagation mechanisms unique to Klinefelter Syndrome remain incompletely understood with a relative paucity of data. Nonetheless, academic interest is increasing in this field as it may further elucidate the pathophysiology behind Klinefelter syndrome.
To perform a systematic review of literature studying the mechanisms of fibrosis initiation or propagation in Klinefelter Syndrome testes.
PubMed was searched systematically for articles specific to Klinefelter Syndrome and the process of fibrosis. Articles that did not contain original data or specifically addressed the target material were excluded. Additional references were extracted when pertinent from the reference lists of included studies.
Primary search yielded 139 articles for abstract review, which was narrowed to 16 for full-text review. Following full-text review, eight contained original data and met topic criteria, with one paper added from reference review for a total of nine papers.
The date range for included papers was 1992-2022. The proposed mechanisms of fibrosis mainly were centered around the impact of altered Sertoli cells on germ cells, the hormonal impact on Leydig cells, the inflammation mediated by mast cells, or the fibrous extracellular matrix deposition by peritubular myoid cells. Additionally, discussions of the role of the altered microvasculature and the specific proteins involved in the blood-testis barrier or the seminiferous tubule architecture are reviewed. Recent papers have incorporated advanced sequencing and offer future directions for targeted gene expression analysis. Still, much of the published data consists solely of immunohistological assessment by age range, creating difficulties in extrapolating causality.
The specific initiating factors of fibrosis of the seminiferous tubules and the propagation mechanisms unique to Klinefelter Syndrome remain incompletely understood with a relative paucity of data. Nonetheless, academic interest is increasing in this field as it may further elucidate the pathophysiology behind Klinefelter syndrome.
摘要：
患有Klinefelter综合征的男性会出现一定程度的生精小管变性，透明质化,和成年后的纤维化。然而,Klinefelter综合征患者睾丸纤维化的病理生理学仍未完全了解.
对研究Klinefelter综合征睾丸纤维化起始或传播机制的文献进行系统综述。
对PubMed进行了系统搜索，以寻找特定于Klinefelter综合征和纤维化过程的文章。不包含原始数据或专门针对目标材料的物品被排除在外。从纳入研究的参考文献列表中提取相关的其他参考文献。
主要搜索产生了139篇摘要评论文章，全文审查范围缩小到16个。在全文审查之后，八个包含原始数据，符合主题标准，从参考文献评论中添加了一篇论文，共9篇论文。
收录论文的日期范围为1992-2022年。提出的纤维化机制主要集中在改变的支持细胞对生殖细胞的影响，荷尔蒙对Leydig细胞的影响，由肥大细胞介导的炎症，或肾小管周围肌样细胞的纤维细胞外基质沉积。此外,对改变的微脉管系统的作用以及与血-睾丸屏障或生精小管结构有关的特定蛋白质的讨论进行了综述。最近的论文已经结合了先进的测序，并为靶向基因表达分析提供了未来的方向。尽管如此，大部分发表的数据仅包括按年龄范围进行的免疫组织学评估，在推断因果关系方面造成困难。
生精小管纤维化的具体启动因素和Klinefelter综合征特有的传播机制仍未完全理解，数据相对缺乏。尽管如此，该领域的学术兴趣正在增加，因为它可能进一步阐明Klinefelter综合征背后的病理生理学。
对研究Klinefelter综合征睾丸纤维化起始或传播机制的文献进行系统综述。
对PubMed进行了系统搜索，以寻找特定于Klinefelter综合征和纤维化过程的文章。
主要搜索产生了139篇摘要评论文章，
收录论文的日期范围为1992-2022年。
生精小管纤维化的具体启动因素和Klinefelter综合征特有的传播机制仍未完全理解，数据相对缺乏。
