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患有先天性上腺增生症的儿童和青少年的淋巴腺变化
Rana Mahmoud1, Marwa Elsayed Abdelrahman2, Hasnaa Hassan Mohamed3
1Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Journal of pediatric endocrinology & metabolism : JPEM
|November 3, 2024
概括
丸上腺休息瘤 (TARTs) 在患有先天性上腺增生症 (CAH) 的男性中很常见. 在男性中查TART至关重要,但在女性中不建议进行常规卵巢成像,除非有卵巢功能障碍.
科学领域:
- 儿科内分泌学 儿科内分泌学
- 生殖内分泌学 生殖内分泌学
- 医疗成像医学成像
背景情况:
- 先天性上腺增生 (CAH) 可以导致男性和女性的淋巴腺功能障碍.
- 丸上腺休息瘤 (TARTs) 是CAH男性不孕症的常见原因.
- 卵巢上腺休息瘤 (OART) 和多囊性卵巢 (PCO) 可以影响CAH女性的卵巢功能.
研究的目的:
- 在被诊断患有CAH的儿童和青少年队列中调查淋巴细胞变化的流行率,特别是TART,OART和PCO.
- 评估TART与临床因素 (如BMI,骨龄和CAH患者的遗传突变) 之间的关系.
主要方法:
- 一项涉及50名21-基酶缺乏症 (21-OHD) CAH患者 (20名男性,30名女性) 的研究,平均年龄为10.35岁.
- 对男性进行丸超声波,对女性进行盆腔MRI.
- 葡萄糖皮质醇剂量和生物化学数据是从医疗记录中收集的.
主要成果:
- 在50%的男性患者中发现了丸上腺休息瘤 (TARTs).
- 在TART和体重指数 (BMI) 标准偏差得分 (SDS) 和骨年龄之间发现了显著的相关性.
- 没有观察到TART与实验室参数,治疗,基因型或特定等位基因之间的显著关联. 只有一个女性表现出多囊性卵巢;没有检测到OART.
结论:
- 高频率的TARTs (50%) 强调了对这些阴道瘤的查在男性的CAH的重要性.
- 在女性患有CAH的患者中,除非存在卵巢功能障碍的症状,否则不适合进行常规卵巢成像.
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