Mendonca Berenice B
Unidade de Endocrinologia do Desenvolvimento, Laboratório de Hormônios e Genética Molecular LIM/42, Disciplina de Endocrinologia, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
Curr Opin Endocrinol Diabetes Obes. 2014 Dec;21(6):511-4. doi: 10.1097/MED.0000000000000115.
To examine the sex assignment in patients with atypical external genitalia, a particularly challenging situation, especially when the genital appearance is not compatible with the sex chromosome.
The most important factors that influence sex assignment include the definite diagnosis, genital appearance, surgical options, potential for fertility, risks of gonadal malignancy and, finally, the perception of the patients and their parents. Full disclosure and complete involvement of the parents in making decisions concerning gender assignment and/or genital surgery must be part of the basic medical care for children with disorder of sex development.
Patients with disorder of sex development should receive long-term care provided by multidisciplinary teams in centers of excellence with ample experience in the management of this disorder.
探讨非典型外生殖器患者的性别指定问题，这是一种特别具有挑战性的情况，尤其是当生殖器外观与性染色体不相符时。
影响性别指定的最重要因素包括明确诊断、生殖器外观、手术选择、生育潜力、性腺恶性肿瘤风险，最后是患者及其父母的认知。充分告知并让父母全面参与有关性别指定和/或生殖器手术的决策，必须成为性发育障碍儿童基本医疗护理的一部分。
性发育障碍患者应由多学科团队在对此类疾病管理经验丰富的卓越中心提供长期护理。
