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克莱恩费尔特综合征与睾丸精子获取结局。

The Klinefelter Syndrome and Testicular Sperm Retrieval Outcomes.

机构信息

Laboratory of Cell Biology, Department of Microscopy, ICBAS-School of Medicine and Biomedical Sciences, University of Porto, Rua Jorge Viterbo Ferreira, 228, 4050-313 Porto, Portugal.

UMIB-Unit for Multidisciplinary Research in Biomedicine, 4050-313 Porto, Portugal.

出版信息

Genes (Basel). 2023 Mar 4;14(3):647. doi: 10.3390/genes14030647.

Abstract

Klinefelter syndrome (KS), caused by the presence of an extra X chromosome, is the most prevalent chromosomal sexual anomaly, with an estimated incidence of 1:500/1000 per male live birth (karyotype 47,XXY). High stature, tiny testicles, small penis, gynecomastia, feminine body proportions and hair, visceral obesity, and testicular failure are all symptoms of KS. Endocrine (osteoporosis, obesity, diabetes), musculoskeletal, cardiovascular, autoimmune disorders, cancer, neurocognitive disabilities, and infertility are also outcomes of KS. Causal theories are discussed in addition to hormonal characteristics and testicular histology. The retrieval of spermatozoa from the testicles for subsequent use in assisted reproduction treatments is discussed in the final sections. Despite testicular atrophy, reproductive treatments allow excellent results, with rates of 40-60% of spermatozoa recovery, 60% of clinical pregnancy, and 50% of newborns. This is followed by a review on the predictive factors for successful sperm retrieval. The risks of passing on the genetic defect to children are also discussed. Although the risk is low (0.63%) when compared to the general population (0.5-1%), patients should be informed about embryo selection through pre-implantation genetic testing (avoids clinical termination of pregnancy). Finally, readers are directed to a number of reviews where they can enhance their understanding of comprehensive diagnosis, clinical care, and fertility preservation.

摘要

克莱恩费尔特综合征(KS)是由额外的 X 染色体引起的,是最常见的染色体性异常,每 1000 个男性活产中估计有 1 例(核型 47,XXY)。高身材、小睾丸、小阴茎、男性乳房发育、女性身体比例和毛发、内脏肥胖和睾丸功能衰竭都是 KS 的症状。内分泌(骨质疏松症、肥胖症、糖尿病)、肌肉骨骼、心血管、自身免疫性疾病、癌症、神经认知障碍和不育也是 KS 的后果。除了激素特征和睾丸组织学外,还讨论了病因理论。最后部分讨论了从睾丸中获取精子以用于后续辅助生殖治疗。尽管睾丸萎缩,生殖治疗仍能取得极好的结果,精子回收率为 40-60%,临床妊娠率为 60%,新生儿率为 50%。接下来是关于成功精子提取预测因素的综述。还讨论了将遗传缺陷遗传给孩子的风险。尽管与一般人群(0.5-1%)相比风险较低(0.63%),但应告知患者通过胚胎植入前遗传检测选择胚胎(避免临床终止妊娠)。最后,读者被引导到一些评论中,他们可以在那里增强对综合诊断、临床护理和生育力保存的理解。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/988d/10048758/c08434fd70ae/genes-14-00647-g001.jpg

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