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46,XY 染色体男性患者受完全性雄激素不敏感综合征或先天性小阴茎影响的长期前景。

Long-term perspectives for 46,XY patients affected by complete androgen insensitivity syndrome or congenital micropenis.

作者信息

Wisniewski Amy B, Migeon Claude J

机构信息

Department of Pediatrics, Division of Pediatric Endocrinology, Johns Hopkins University School of Medicine, 600 N. Wolfe Street/Park 211, Baltimore, MD 21287, USA.

出版信息

Semin Reprod Med. 2002 Aug;20(3):297-304. doi: 10.1055/s-2002-35376.

Abstract

Controversy concerning optimal treatment for individuals affected by syndromes of abnormal sex differentiation can best be resolved with knowledge about long-term medical, surgical, and psychosexual outcomes of patients. Follow-up information has recently been gathered on older cohorts of the following patient groups: (1) those affected by complete androgen insensitivity syndrome (CAIS) raised female and (2) those affected by congenital micropenis raised male or female. As a group, women with CAIS were satisfied with their female gender and sexual function. However, a need for better patient education was identified for this specific population. Most patients with congenital micropenis, whether raised male or female, were satisfied with their gender. Regardless of sex of rearing, dissatisfaction with the appearance and function of the genitalia as judged by both physicians and subjects was evident. For patients with congenital micropenis, male sex of rearing was concluded to be optimal because genital reconstructive surgery is not required with this choice.

摘要

关于性发育异常综合征患者的最佳治疗方法的争议,最好通过了解患者的长期医学、手术和心理性结果来解决。最近收集了以下患者群体较年长队列的随访信息:(1)那些患有完全雄激素不敏感综合征(CAIS)且被抚养为女性的患者,以及(2)那些患有先天性小阴茎且被抚养为男性或女性的患者。总体而言,患有CAIS的女性对自己的女性性别和性功能感到满意。然而,已确定这一特定人群需要更好的患者教育。大多数先天性小阴茎患者,无论被抚养为男性还是女性,都对自己的性别感到满意。无论抚养性别如何,医生和患者都明显对生殖器的外观和功能不满意。对于先天性小阴茎患者,得出的结论是抚养为男性是最佳选择,因为这种选择不需要进行生殖器重建手术。

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