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双侧睾丸切除术治疗完全性雄激素不敏感综合征:随访1年的患者结局

Bilateral orchiectomy for the surgical treatment of complete androgen insensitivity syndrome: patient outcome after 1 year of follow-up.

作者信息

Sills E Scott, Perloe Mark, Kaplan Carolyn R, Schlegel Peter N, Palermo Gianpiero D

机构信息

Reproductive Specialists LLC, Division of Reproductive Endocrinology and Infertility, Department of Obstetrics and Gynecology, Atlanta Medical Center, Atlanta, Georgia 30342, USA.

出版信息

J Laparoendosc Adv Surg Tech A. 2003 Jun;13(3):193-7. doi: 10.1089/109264203766207735.

DOI:10.1089/109264203766207735
PMID:12855103
Abstract

OBJECTIVE

To describe an operative approach to bilateral orchiectomy for complete androgen insensitivity syndrome (AIS) and to report patient status 1 year after surgery.

MATERIAL AND METHOD

A 19(1/2)-year-old woman with primary amenorrhea, cervical/uterine agenesis, elevated serum testosterone, and 46,XY karyotype underwent characterization of the androgen receptor mutation by polymorphism sequence analysis. Bilateral orchiectomy was performed via a triple-puncture 5-mm laparoscope, and postsurgical hormone replacement followed a daily oral contraceptive model. Psychological counseling was provided to the patient and her family.

RESULTS

Minimally invasive removal of the gonads was accomplished without complication. Microscopic examination of the testes revealed a benign Sertoli cell-only pattern. After 1 year, the patient's level of functioning and psychosexual status were unchanged in comparison with before surgery.

CONCLUSIONS

Patients with complete AIS should undergo postpubertal gonadectomy because of an increased risk for malignant transformation of the testicles, and this can be performed safely via small-caliber laparoscopy. Attention to psychological considerations in such patients is important to maximize long-term success.

摘要

目的

描述完全性雄激素不敏感综合征(AIS)双侧睾丸切除术的手术方法,并报告术后1年患者的状况。

材料与方法

一名19岁半的女性,原发性闭经,宫颈/子宫发育不全,血清睾酮升高,核型为46,XY,通过多态性序列分析对雄激素受体突变进行了鉴定。通过三孔5毫米腹腔镜进行双侧睾丸切除术,术后激素替代遵循每日口服避孕药模式。为患者及其家人提供了心理咨询。

结果

以微创方式成功切除性腺,无并发症。睾丸的显微镜检查显示为仅支持细胞的良性模式。1年后,患者的功能水平和心理性状态与手术前相比没有变化。

结论

由于睾丸恶性转化风险增加,完全性AIS患者应在青春期后进行性腺切除术,并且可以通过小口径腹腔镜安全地进行。关注此类患者的心理因素对于实现长期成功很重要。

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Bilateral orchiectomy for the surgical treatment of complete androgen insensitivity syndrome: patient outcome after 1 year of follow-up.双侧睾丸切除术治疗完全性雄激素不敏感综合征:随访1年的患者结局
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