Research Group Biology of Testis, Department of Embryology and Genetics, Vrije Universiteit Brussel, Laarbeeklaan 103, 1090 Brussels, Belgium.
Hum Reprod. 2012 Feb;27(2):323-30. doi: 10.1093/humrep/der425. Epub 2011 Dec 12.
Although early development of testes appears normal in boys with Klinefelter syndrome (KS), spermatogonial stem cell (SSC) depletion occurs in mid puberty, leading to infertility. Cryopreservation of SSCs prior to stem cell loss is an option that is currently offered to boys who have to undergo gonadotoxic treatments. This study aimed to explore the possibility of preserving SSCs in pubertal KS adolescents by testicular tissue banking.
A retrospective study was conducted in seven non-mosaic 47,XXY adolescents, aged 13-16 years, who were invited for an experimental testicular tissue banking programme during their follow-up at the Paediatric Endocrinology Department of the UZ Brussel between 2009 and 2011. Paraffin-embedded testicular tissue was sectioned and stained with haematoxylin-eosin, and immunostainings were performed for Mage-A4, anti-Mullerian hormone, Inhibin α and steroidogenic acute regulatory protein. The presence of spermatogenesis and/or spermatogonia was evaluated.
Massive fibrosis and hyalinization was observed in all but one KS patients. Although spermatogonia were seen in five patients, spermatogonia were only present in tubules showing normal architecture in the youngest patient who also had normal follicle-stimulating hormone and inhibin B concentrations.
Testicular tissue cryopreservation in KS adolescents should be recommended as soon as possible, probably before hormonal changes of failing Sertoli cell function are detected.
尽管克氏综合征(KS)男孩的睾丸早期发育似乎正常,但精原干细胞(SSC)在青春期中期会耗竭,导致不育。在干细胞丢失之前冷冻保存 SSC 是一种选择,目前向必须接受性腺毒性治疗的男孩提供这种选择。本研究旨在通过睾丸组织库探索在青春期 KS 青少年中保存 SSC 的可能性。
对 2009 年至 2011 年期间在布鲁塞尔 UZ 儿科内分泌科随访的 7 名非嵌合 47,XXY 青春期前男孩进行了回顾性研究,他们受邀参加了实验性睾丸组织库计划。对石蜡包埋的睾丸组织进行切片,并用苏木精-伊红染色,并用 Mage-A4、抗苗勒管激素、抑制素 α 和类固醇生成急性调节蛋白进行免疫染色。评估生精和/或精原细胞的存在情况。
除 1 名 KS 患者外,所有患者均观察到大量纤维化和玻璃样变。尽管在 5 名患者中观察到精原细胞,但在最年轻的患者中,只有在显示正常结构的小管中才存在精原细胞,该患者的卵泡刺激素和抑制素 B 浓度也正常。
KS 青少年的睾丸组织冷冻保存应尽快推荐,可能在检测到 Sertoli 细胞功能衰竭的激素变化之前。