Department of Urology, Wake Forest School of Medicine, Winston-Salem, North Carolina, USA.
Wake Forest Institute for Regenerative Medicine (WFIRM), Wake Forest School of Medicine, Winston-Salem, North Carolina, USA.
Andrology. 2023 Mar;11(3):435-443. doi: 10.1111/andr.13327. Epub 2022 Oct 27.
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.
MATERIALS/METHODS: 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.
患有克莱恩费尔特综合征的男性成年后会出现一定程度的生精小管退化、玻璃样变和纤维化。然而，克莱恩费尔特综合征患者睾丸纤维化的病理生理学仍不完全清楚。
对研究克莱恩费尔特综合征睾丸纤维化起始或进展机制的文献进行系统回顾。
材料/方法：系统地在 PubMed 上搜索与克莱恩费尔特综合征和纤维化过程相关的文章。排除未包含原始数据或专门针对目标材料的文章。从纳入研究的参考文献中提取相关的额外参考文献。
初步搜索得到 139 篇摘要进行审查，缩小至 16 篇进行全文审查。在全文审查后，有 8 篇包含原始数据并符合主题标准，另有 1 篇从参考文献审查中添加，共 9 篇论文。
纳入文献的日期范围为 1992 年至 2022 年。纤维化的提出机制主要集中在改变的支持细胞对生殖细胞的影响、激素对间质细胞的影响、肥大细胞介导的炎症，或由小管周肌样细胞引起的纤维细胞外基质沉积。此外，还讨论了改变的微血管和参与血睾屏障或生精小管结构的特定蛋白的作用。最近的论文采用了先进的测序技术，并为靶向基因表达分析提供了未来的方向。尽管如此，大部分已发表的数据仅包含按年龄范围进行的免疫组织化学评估，这使得推断因果关系变得困难。
生精小管纤维化的特定起始因素和克莱恩费尔特综合征特有的传播机制仍不完全清楚，相关数据相对较少。尽管如此，由于该领域可能进一步阐明克莱恩费尔特综合征背后的病理生理学，学术界对其兴趣正在增加。
