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在一名患有严重少精子症的男性先天性肾上腺皮质增生症患者中,通过更换类固醇改善精液参数。

Improvement in semen parameters by switching steroids in a male congenital adrenal hyperplasia patient with severe oligozoospermia.

作者信息

Onishi Atsushi, Okada Keisuke, Sato Katsuya, Kaku Yasuhiro, Chiba Koji, Fujisawa Masato

机构信息

Division of Urology, Department of Surgery Related, Kobe University Graduate School of Medicine, Kobe, Japan, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, 6500017, Japan.

出版信息

Urol Case Rep. 2023 Mar 1;47:102368. doi: 10.1016/j.eucr.2023.102368. eCollection 2023 Mar.

DOI:10.1016/j.eucr.2023.102368
PMID:36915704
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10006705/
Abstract

Congenital adrenal hyperplasia (CAH) causes hypogonadotropic hypogonadism due to the excessive production of adrenal androgens, which results in hypospermatogenesis in some male patients. We herein present a CAH case with hypogonadotropic hypogonadism and male infertility. A 26-year-old male receiving steroid therapy for 21 hydroxylase deficiency was diagnosed with low gonadotropin levels, an elevated ACTH level, and severe oligozoospermia. The switching from hydrocortisone to dexamethasone resulted in the normalization of gonadotropin levels and semen findings. The couple underwent ICSI-ET, resulting in a live birth. In cases of CAH with hypospermatogenesis, the continuous suppression of ACTH by dexamethasone may restore spermatogenesis.

摘要

先天性肾上腺皮质增生症(CAH)由于肾上腺雄激素分泌过多导致低促性腺激素性性腺功能减退,这在一些男性患者中会导致精子发生减少。我们在此报告一例患有低促性腺激素性性腺功能减退和男性不育症的CAH病例。一名因21-羟化酶缺乏接受类固醇治疗的26岁男性被诊断为促性腺激素水平低、促肾上腺皮质激素(ACTH)水平升高和严重少精子症。从氢化可的松换用地塞米松后,促性腺激素水平和精液检查结果恢复正常。这对夫妇接受了卵胞浆内单精子注射-胚胎移植(ICSI-ET),并成功分娩。在患有精子发生减少的CAH病例中,地塞米松持续抑制ACTH可能会恢复精子发生。

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本文引用的文献

1
Congenital Adrenal Hyperplasia-Current Insights in Pathophysiology, Diagnostics, and Management.先天性肾上腺皮质增生症-病理生理学、诊断和治疗的最新见解。
Endocr Rev. 2022 Jan 12;43(1):91-159. doi: 10.1210/endrev/bnab016.
2
Two cases of male patients followed for a classical form of congenital adrenal hyperplasia (CAH), presenting an azoospermia: analysis and review of the literature.两例患有典型先天性肾上腺皮质增生症(CAH)且表现为无精子症的男性患者:病例分析及文献综述
Basic Clin Androl. 2019 Mar 12;29:10. doi: 10.1186/s12610-019-0084-8. eCollection 2019.
3
Experience in optimizing fertility outcomes in men with congenital adrenal hyperplasia due to 21 hydroxylase deficiency.优化因21-羟化酶缺乏所致先天性肾上腺皮质增生症男性患者生育结局的经验。
Clin Endocrinol (Oxf). 2016 Jun;84(6):830-6. doi: 10.1111/cen.13001. Epub 2016 Feb 15.
4
Repeated successful induction of fertility after replacing hydrocortisone with dexamethasone in a patient with congenital adrenal hyperplasia and testicular adrenal rest tumors.在一名患有先天性肾上腺皮质增生症和睾丸肾上腺残余肿瘤的患者中,用 dexamethasone 替代 hydrocortisone 后反复成功诱导生育。
Fertil Steril. 2007 Sep;88(3):705.e5-8. doi: 10.1016/j.fertnstert.2006.11.148. Epub 2007 May 22.
5
Primary infertility in 45-year-old man with untreated 21-hydroxylase deficiency: successful outcome with glucocorticoid therapy.一名45岁未经治疗的21-羟化酶缺乏症男性的原发性不孕症:糖皮质激素治疗取得成功结果。
J Clin Endocrinol Metab. 2002 Jun;87(6):2442-5. doi: 10.1210/jcem.87.6.8616.