Abstract：
OBJECTIVE: Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder characterized by impaired activity of the enzyme required for cortisol and aldosterone production, resulting in increased adrenal androgen synthesis. Factors affecting fertility in CAH patients include ambiguous genitalia and their complications, excessive androgen secretion, adrenal progesterone hypersecretion, and various psychosocial factors. Serum anti-Müllerian hormone (AMH) level is used to assess ovarian reserve in women. A few data on serum AMH levels in CAH patients are available in the literature. The aim of the study was to evaluate ovarian reserve in a group of post-menarche females diagnosed with CAH by measuring serum AMH level and assessing the number of antral follicles sonographically.
METHODS: A case-control study was conducted on 17 post-pubertal CAH females and 17 age-matched healthy female controls; the mean age of the patient group was 15.09 ± 3.55 years ranging from 11 to 24 years, while the mean age of the control group was 16.04 ± 3.72 years ranging from 12 to 25 years, the mean post-menarchal age of the patients group was 3.29 ± 1.37 years ranging from 1 to 6 years while the mean post-menarchal age of the control group was 4.13 ± 1.62 years ranging from 1 to 9 years. The degree of hirsutism was compared between the two groups according to the modified Ferriman-Gallwey score, clitoral length was assessed using a digital caliber. Serum levels of adrenal androgens in addition to basal levels of serum follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol, progesterone, and serum AMH were measured in both groups.
RESULTS: Patients had smaller uterine volumes, and smaller ovarian volumes but a comparable number of antral follicles and comparable serum AMH levels relative to controls.
CONCLUSIONS: Good compliance with treatment in patients with CAH results in good hormonal control, low risk of PCOS, good fertility parameters, and a good ovarian reserve.
METHODS: A case-control study was conducted on 17 post-pubertal CAH females and 17 age-matched healthy female controls; the mean age of the patient group was 15.09 ± 3.55 years ranging from 11 to 24 years, while the mean age of the control group was 16.04 ± 3.72 years ranging from 12 to 25 years, the mean post-menarchal age of the patients group was 3.29 ± 1.37 years ranging from 1 to 6 years while the mean post-menarchal age of the control group was 4.13 ± 1.62 years ranging from 1 to 9 years. The degree of hirsutism was compared between the two groups according to the modified Ferriman-Gallwey score, clitoral length was assessed using a digital caliber. Serum levels of adrenal androgens in addition to basal levels of serum follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol, progesterone, and serum AMH were measured in both groups.
RESULTS: Patients had smaller uterine volumes, and smaller ovarian volumes but a comparable number of antral follicles and comparable serum AMH levels relative to controls.
CONCLUSIONS: Good compliance with treatment in patients with CAH results in good hormonal control, low risk of PCOS, good fertility parameters, and a good ovarian reserve.
摘要：
背景：先天性肾上腺增生（CAH）是一种常染色体隐性遗传疾病，其特征是皮质醇和醛固酮产生所需的酶活性受损，导致肾上腺雄激素合成增加。影响CAH患者生育能力的因素包括生殖器模糊及其并发症，雄激素分泌过多,肾上腺孕酮分泌过多，和各种社会心理因素。血清抗苗勒管激素（AMH）水平用于评估女性的卵巢储备。文献中提供了一些关于CAH患者血清AMH水平的数据。
目的：通过测量血清AMH水平和超声评估窦卵泡数量来评估月经初潮后诊断为CAH的女性的卵巢储备。
方法：对17名青春期后CAH女性和17名年龄匹配的健康女性对照进行了病例对照研究；患者组的平均年龄为15.09±3.55岁，范围为11至24岁，而对照组的平均年龄为16.04±3.72岁，范围为12至25岁，患者组的平均初潮后年龄为3.29±1.37岁，范围为1至6岁，而对照组的平均初潮后年龄为4.13±1.62岁，范围为1至9岁。根据改良的Ferriman-Gallwey评分比较两组多毛症程度,使用数字口径评估阴蒂长度。除了基础水平的血清卵泡刺激素（FSH），肾上腺雄激素的血清水平，黄体生成素(LH),雌二醇,黄体酮,检测两组血清抗苗勒管激素(AMH)水平。超声评估子宫体积，卵巢体积,和窦卵泡的数量。
结果：患者的子宫体积较小，与对照组相比，卵巢体积较小，但窦卵泡数量相当，血清AMH水平相当。
结论：CAH患者良好的治疗依从性导致激素控制良好，PCOS的低风险，良好的生育参数，和良好的卵巢储备.
目的：通过测量血清AMH水平和超声评估窦卵泡数量来评估月经初潮后诊断为CAH的女性的卵巢储备。
方法：对17名青春期后CAH女性和17名年龄匹配的健康女性对照进行了病例对照研究；患者组的平均年龄为15.09±3.55岁，范围为11至24岁，而对照组的平均年龄为16.04±3.72岁，范围为12至25岁，患者组的平均初潮后年龄为3.29±1.37岁，范围为1至6岁，而对照组的平均初潮后年龄为4.13±1.62岁，范围为1至9岁。根据改良的Ferriman-Gallwey评分比较两组多毛症程度,使用数字口径评估阴蒂长度。除了基础水平的血清卵泡刺激素（FSH），肾上腺雄激素的血清水平，黄体生成素(LH),雌二醇,黄体酮,检测两组血清抗苗勒管激素(AMH)水平。超声评估子宫体积，卵巢体积,和窦卵泡的数量。
结果：患者的子宫体积较小，与对照组相比，卵巢体积较小，但窦卵泡数量相当，血清AMH水平相当。
结论：CAH患者良好的治疗依从性导致激素控制良好，PCOS的低风险，良好的生育参数，和良好的卵巢储备.
