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患有 21-羟化酶缺陷的经典先天性肾上腺皮质增生症女性的雄激素过多与骨密度的关系。

Association of androgen excess and bone mineral density in women with classical congenital adrenal hyperplasia with 21-hydroxylase deficiency.

机构信息

Department of Internal Medicine, Seoul National University Hospital, Seoul, South Korea.

Department of Internal Medicine, Seoul National University College of Medicine, 101 Dae-hak ro, Jongno gu, Seoul, 03080, Korea.

出版信息

Arch Osteoporos. 2022 Mar 8;17(1):45. doi: 10.1007/s11657-022-01090-0.

DOI:10.1007/s11657-022-01090-0
PMID:35258698
Abstract

UNLABELLED

The relationship between androgen excess and bone health in patients with congenital adrenal hyperplasia (CAH) with 21-hydroxylase (21-OH) deficiency is not fully understood. This study demonstrated positive correlations between androgen hormones and bone mineral density (BMD) in CAH women with 21-OH deficiency.

PURPOSE

This study aims to assess BMD and its association with androgen excess in women with CAH.

METHODS

We enrolled 92 women with CAH with 21-OH deficiency and retrospectively reviewed their clinical features, hormone concentrations, body composition, glucocorticoid (GC) dose, and BMD.

RESULTS

BMD was not different according to the subtypes of CAH. BMD at the lumbar spine was lower in women with CAH with regular menstruation than those with irregular menstruation (1.081 vs. 1.165 g/cm, P < 0.05). BMD was lower in women with CAH with 17-hydroxyprogesterone (17-OHP) < 10 ng/mL than in those with ≥ 10 ng/mL (lumbar spine, 1.019 vs. 1.150 g/cm; femur neck, 0.806 vs. 0.899 g/cm; total hip, 0.795 vs. 0.943 g/cm; all P < 0.05). After adjusting for age and BMI in correlation analyses, testosterone concentrations were positively correlated with lumbar spine, femur neck, and total hip BMD (r = 0.46, r = 0.38, and r = 0.35, respectively; all P < 0.05), while 17-OHP was positively correlated with lumbar spine BMD (r = 0.38, P < 0.01). In subgroup analysis, 17-OHP was positively correlated with BMD (lumbar spine, r = 0.22; femur neck, r = 0.22; total hip, r = 0.24; all P < 0.05) only in the group with a total cumulative dose of GC ≥ 156.0 g/m.

CONCLUSION

Androgen excess may have a protective effect on BMD in women with classic CAH and high cumulative doses of GC.

摘要

目的

本研究旨在评估先天性肾上腺皮质增生症(CAH)女性的骨密度(BMD)及其与雄激素过多的关系。

方法

我们招募了 92 名患有 21-羟化酶(21-OH)缺乏症的 CAH 女性,并回顾性分析了她们的临床特征、激素浓度、体成分、糖皮质激素(GC)剂量和 BMD。

结果

BMD 与 CAH 的亚型无关。月经规律的 CAH 女性腰椎 BMD 低于月经不规律的女性(1.081 比 1.165 g/cm,P<0.05)。17-羟孕酮(17-OHP)<10 ng/mL 的 CAH 女性腰椎 BMD 低于≥10 ng/mL 的女性(腰椎,1.019 比 1.150 g/cm;股骨颈,0.806 比 0.899 g/cm;全髋,0.795 比 0.943 g/cm;均 P<0.05)。在相关分析中,校正年龄和 BMI 后,睾酮浓度与腰椎、股骨颈和全髋 BMD 呈正相关(r=0.46、r=0.38 和 r=0.35;均 P<0.05),而 17-OHP 与腰椎 BMD 呈正相关(r=0.38,P<0.01)。在亚组分析中,17-OHP 与 BMD 呈正相关(腰椎,r=0.22;股骨颈,r=0.22;全髋,r=0.24;均 P<0.05),仅在 GC 总累积剂量≥156.0 g/m 的组中如此。

结论

雄激素过多可能对接受大剂量 GC 治疗的经典 CAH 女性的 BMD 有保护作用。

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Long-Term Health Outcomes of Korean Adults With Classic Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency.经典 21-羟化酶缺陷型先天性肾上腺皮质增生症韩国成年患者的长期健康结局
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Clinical and Hormonal Profiles Correlate With Molecular Characteristics in Patients With 11β-Hydroxylase Deficiency.临床和激素特征与 11β-羟化酶缺陷患者的分子特征相关。
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