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成年经典型先天性肾上腺皮质增生症女性患者的长期皮质类固醇替代治疗与骨矿物质密度

Long-term corticosteroid replacement and bone mineral density in adult women with classical congenital adrenal hyperplasia.

作者信息

King Jeremy A, Wisniewski Amy B, Bankowski Brandon J, Carson Kathryn A, Zacur Howard A, Migeon Claude J

机构信息

Division of Reproductive Endocrinology, Department of Gynecology and Obstetrics, Johns Hopkins School of Medicine, Baltimore, Maryland 21287, USA.

出版信息

J Clin Endocrinol Metab. 2006 Mar;91(3):865-9. doi: 10.1210/jc.2005-0745. Epub 2005 Nov 8.

Abstract

CONTEXT

Concern has been raised regarding the potential impact of chronic glucocorticoid therapy on the bone mineral density (BMD) of patients with congenital adrenal hyperplasia (CAH).

OBJECTIVE

The purpose of this investigation was to assess the impact of chronic glucocorticoid replacement in adult women with classical CAH.

PATIENTS AND DESIGN

We used dual energy x-ray absorptiometry to evaluate lumbar spine and whole body BMD in 11 women with salt-losing (SL) CAH and 15 with the simple virilizing form. Physical characteristics and serum hormone concentrations were also measured. Results were compared with those of unaffected sisters of CAH patients (n = 9).

MAIN OUTCOME MEASURE

BMD was the main outcome measure.

RESULTS

Osteopenia was noted in 45% of SL CAH patients, 13% of patients with the simple virilizing form, and 11% of controls. Lumbar spine and whole body BMDs of CAH subjects were lower than those of controls (P < 0.05). Compared with CAH subjects with normal BMD, those with osteopenia had reduced serum levels of dehydroepiandrosterone sulfate and dehydroepiandrosterone. Adrenal androgen levels were particularly suppressed among postmenopausal women receiving glucocorticoid replacement.

CONCLUSIONS

Adult women with classical CAH treated with long-term glucocorticoids are at risk for decreased BMD, especially those with the SL form. Oversuppression of adrenal androgens is associated with increased risk for bone loss in this population.

摘要

背景

慢性糖皮质激素治疗对先天性肾上腺皮质增生症(CAH)患者骨矿物质密度(BMD)的潜在影响已引起关注。

目的

本研究旨在评估成年女性经典型CAH患者长期糖皮质激素替代治疗的影响。

患者与设计

我们采用双能X线吸收法评估了11例失盐型(SL)CAH女性患者和15例单纯男性化型患者的腰椎和全身骨密度。还测量了身体特征和血清激素浓度。将结果与CAH患者未受影响的姐妹(n = 9)的结果进行比较。

主要观察指标

骨密度是主要观察指标。

结果

45%的失盐型CAH患者、13%的单纯男性化型患者和11%的对照者存在骨质减少。CAH患者的腰椎和全身骨密度低于对照组(P < 0.05)。与骨密度正常的CAH患者相比,骨质减少的患者血清硫酸脱氢表雄酮和脱氢表雄酮水平降低。在接受糖皮质激素替代治疗的绝经后女性中,肾上腺雄激素水平尤其受到抑制。

结论

长期接受糖皮质激素治疗的成年女性经典型CAH患者存在骨密度降低的风险,尤其是失盐型患者。肾上腺雄激素过度抑制与该人群骨质流失风险增加有关。

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