PURPOSE Gender assignment represents one of the most controversial aspects of the clinical management of individuals with Differences of Sex Development, including 5α-Reductase-2 deficiency (SRD5A2). Given the predominant female appearance of external genitalia in individuals with SRD5A2 deficiency, most of them were assigned to the female sex at birth. However, in the last years the high rate of gender role shift from female to male led to recommend a male gender assignment. METHODS We here describe two cases of subjects with SRD5A2 deficiency assigned as females at birth, reporting their clinical histories and psychometric evaluations (Body Uneasiness Test, Utrecht Gender Dysphoria Scale, Bem Sex-Role Inventory, Female Sexual Distress Scale Revised, visual analogue scale for gender identity and sexual orientation) performed at the time of referral at the Florence Gender Clinic. RESULTS Both patients underwent early surgical interventions without being included in the decision-making process. They had to conform to a binary feminine gender role because of social/familiar pressure, with a significant impact on their psychological well-being. Psychometric evaluations identified clinically significant body uneasiness and gender incongruence in both subjects. No sexually related distress and undifferentiated gender role resulted in the first subject and sexually related distress and androgynous gender role resulted in the second subject. CONCLUSIONS The reported cases suggest the possibility to consider a new approach for gender assignment in these individuals, involving them directly in the decision-making process and allowing them to explore their gender identity, also with the help of GnRH analogues to delay pubertal modifications.

中文翻译：

---

5α-还原酶2缺乏症：婴儿期是否建议进行性别分配？两次病例报告和文献复习。

目的性别分配代表具有性别发展差异（包括5α-还原酶-2缺乏症（SRD5A2））的个体临床管理中最具争议的方面之一。鉴于SRD5A2缺乏症患者外生殖器中女性占主导地位，因此大多数人在出生时就被分配为女性。然而，在最近几年中，性别角色从女性转移到男性的比率很高，因此建议推荐一个男性性别任务。方法我们在这里描述了两例患有SRD5A2缺乏症的受试者，她们在出生时被分配为女性，报告了他们的临床历史和心理测评（身体耐性测试，乌得勒支性别焦虑量表，Bem性角色量表，女性性苦恼量表修订版，在佛罗伦萨性别诊所转诊时进行的视觉模拟量表，以了解性别认同和性取向）。结果两名患者均接受了早期外科手术，未纳入决策过程。由于社会/家庭压力，他们不得不遵循双重女性性别角色，对他们的心理健康产生重大影响。心理测验评估确定了两个受试者在临床上均存在明显的身体不适和性别不一致。没有性相关的困扰和性别角色未分化导致第一个主题，而性相关的困扰和雌雄同体的性别角色导致第二个主题。结论所报告的病例表明，有可能考虑对这些人进行性别分配的新方法，