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因CYP21缺乏导致经典型先天性肾上腺皮质增生症女孩的骨矿物质密度

Bone mineral density in girls with classical congenital adrenal hyperplasia due to CYP21 deficiency.

作者信息

Elnecave Regina Helena, Kopacek Cristiane, Rigatto Marilia, Keller Brenner Juliana, Sisson de Castro José Augusto

机构信息

Hospital de Clinicas de Porto Alegre, Endocrinology Section, Porto Alegre, Brasil.

出版信息

J Pediatr Endocrinol Metab. 2008 Dec;21(12):1155-62. doi: 10.1515/jpem.2008.21.12.1155.

Abstract

AIM

To verify possible associations among glucocorticoid doses, use of dexamethasone, and bone mineral density (BMD), measured by dual X-ray absorptiometry (DXA) and quantitative computed tomography (QCT), in female children with congenital adrenal hyperplasia due to CYP21 deficiency (CAH-CYP21). Classical CAH-CYP21 in females allows the study of the effects of hyperandrogenism and chronic glucocorticoid exposure.

DESIGN

Cross-sectional observational study.

PATIENTS

Sixteen girls (4-19 years) with CAH-CYP21 and 32 age-matched control girls.

MEASUREMENTS

BMD was the main outcome measure assessed by total body and lumbar spine L1-L4 DXA (DXAtot and DXAIs), lumbar spine L1-L4 bone mineral apparent density (BMAD) and spinal L1-L4 QCT of trabecular (QCTtrab) and cortical (QCTcort) bone. The glucocorticoid dose used by patients with CAH-CYP21 was expressed as hydrocortisone equivalents/m2.

RESULTS

Mean BMD in both groups was similar by any method. In patients, BMD decreased with the increasing mean dose of glucocorticoid, seen in QCTcort (r = -0.55; p = 0.03) and QCTtrab (r = -0.52; p = 0.04). There was also a negative correlation between cumulative glucocorticoid dose and BMD in QCTcort (beta = -0.0016; p = 0.005) and QCTtrab (beta = -0.0009; p = 0.03).

CONCLUSIONS

The dose of glucocorticoid used in the treatment of girls with CAH-CYP21 correlated negatively with BMD, and dexamethasone was not selectively harmful.

摘要

目的

验证因CYP21缺乏所致先天性肾上腺皮质增生症(CAH-CYP21)女童中,通过双能X线吸收测定法(DXA)和定量计算机断层扫描(QCT)测量的糖皮质激素剂量、地塞米松的使用与骨密度(BMD)之间可能存在的关联。女性经典型CAH-CYP21有助于研究高雄激素血症和慢性糖皮质激素暴露的影响。

设计

横断面观察性研究。

患者

16名患有CAH-CYP21的女孩(4 - 19岁)和32名年龄匹配的对照女孩。

测量

骨密度是主要结局指标,通过全身和腰椎L1 - L4的DXA(DXAtot和DXAIs)、腰椎L1 - L4骨矿物质表观密度(BMAD)以及腰椎L1 - L4小梁骨(QCTtrab)和皮质骨(QCTcort)的QCT进行评估。CAH-CYP21患者使用的糖皮质激素剂量以氢化可的松等效剂量/m²表示。

结果

两组的平均骨密度通过任何方法测量均相似。在患者中,骨密度随着糖皮质激素平均剂量的增加而降低,在QCTcort(r = -0.55;p = 0.03)和QCTtrab(r = -0.52;p = 0.04)中可见。QCTcort(β = -0.0016;p = 0.005)和QCTtrab(β = -0.0009;p = 0.03)中累积糖皮质激素剂量与骨密度之间也存在负相关。

结论

用于治疗CAH-CYP21女童的糖皮质激素剂量与骨密度呈负相关,且地塞米松并无选择性危害。

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