在男孩部分雄激素不敏感综合征的妇及其管理
Supitcha Patjamontri1,2, Angela K Lucas-Herald1, Jillian Bryce1
1Developmental Endocrinology Research Group, University of Glasgow, Royal Hospital for Children, Glasgow G51 4TF, UK.
The Journal of clinical endocrinology and metabolism
|August 30, 2024
概括
妇在患有部分雄激素不敏感综合征 (PAIS) 的男性中很常见,但并非普遍存在. 外科治疗似乎比治疗PAIS相关性妇产不良的药物治疗更有效.
科学领域:
- 内分泌学 在内分泌学.
- 遗传学 是一个遗传学.
- 生殖健康 生殖健康
背景情况:
- 部分雄激素不敏感综合征 (PAIS) 是一种罕见的疾病.
- 在患有PAIS的男性中,经常观察到妇.
研究的目的:
- 评估被诊断患有PAIS的男性妇产不良的管理策略.
- 为了比较PAIS患者妇产不良的医疗和手术干预措施的有效性.
主要方法:
- 来自I-DSD注册表的10岁以上男性PAIS患者的回顾性审查.
- 分析了来自57个符合条件的病例的数据,这些病例中确实存在雄激素受体基因变异.
主要成果:
- 在基因确诊的PAIS病例中,91%的病例中出现了妇,中位发病时间为13.5年.
- 与药物治疗 (中位数3) 相比,手术治疗的有效性得分明显更高 (中位数为10).
- 在77%的患有妇产不良的病例中观察到微阴茎;自发解消很少 (6%).
结论:
- 妇是PAIS中非常普遍的疾病,尽管并非普遍存在.
- 手术干预通常比医疗治疗更有效地管理PAIS中的妇产不良.
- 需要进行进一步的标准化研究,以优化PAIS妇产管理.
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