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13号染色体镶嵌三体与青春期体质性发育延迟

Mosaic trisomy 13 and constitutional delay in puberty.

作者信息

Eltayeb Sara Yousif Hussein, Bashier Alaaeldin, Hussain Azza Abdulaziz Khalifa Bin

机构信息

Internal Medicine Department, Rashid Hospital, P.O Box 4545, Dubai, United Arab Emirates.

Endocrinology Department, Dubai Hospital, Dubai, United Arab Emirates.

出版信息

Oxf Med Case Reports. 2022 May 23;2022(5):omac046. doi: 10.1093/omcr/omac046. eCollection 2022 May.

Abstract

Patau syndrome is the third most frequent chromosomal trisomy, with an estimated mortality rate that is about 50 times higher than the general neonatal mortality rate. Trisomy 13 mosaicism is a subtype that may result in a milder form of the disease, potentially leading to a longer life expectancy in these patients, allowing them to reach puberty. In this report, we discuss the case of a young boy who was evaluated for delayed puberty and karyotyping revealed mosaicism for trisomy 13. A detailed history, physical examination and appropriate laboratory studies and imaging were performed that showed a picture of suggestive of hypogonadotropic hypogonadism and an abnormal male karyotype with mosaicism for trisomy 13. To date, there is no clear explanation to the association between trisomy 13 and gonadal axis, specifically with normal imaging of pituitary. We postulate that delayed puberty could be a clinical feature of mosaic trisomy 13.

摘要

帕陶综合征是第三常见的染色体三体疾病,其估计死亡率约为新生儿总体死亡率的50倍。13三体嵌合体是一种亚型,可能导致病情较轻,这些患者的预期寿命可能更长,甚至能够进入青春期。在本报告中,我们讨论了一名因青春期发育延迟接受评估的小男孩的病例,核型分析显示为13三体嵌合体。我们进行了详细的病史询问、体格检查以及适当的实验室检查和影像学检查,结果显示为低促性腺激素性性腺功能减退,男性核型异常,存在13三体嵌合体。迄今为止,对于13三体与性腺轴之间的关联,特别是垂体影像学正常的情况下,尚无明确解释。我们推测青春期发育延迟可能是13三体嵌合体的一个临床特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3ce4/9127954/8fd07e8ec432/omac046f1.jpg

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