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

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.

• Authors: Regina Helena Elnecave, Cristiane Kopacek, Marilia Rigatto, Juliana Keller Brenner, José Augusto Sisson de Castro
• Journal: Journal of pediatric endocrinology & metabolism : JPEM (J Pediatr Endocrinol Metab) Vol. 21 Issue 12 Pg. 1155-62 (Dec 2008) ISSN: 0334-018X [Print] Germany
• PMID: 19189689 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Glucocorticoids
• Dexamethasone
• CYP21A2 protein, human
• Steroid 21-Hydroxylase

Topics

• Absorptiometry, Photon
• Adolescent
• Adrenal Hyperplasia, Congenital (drug therapy, genetics, physiopathology)
• Body Mass Index
• Bone Density (drug effects, physiology)
• Case-Control Studies
• Child
• Child, Preschool
• Cross-Sectional Studies
• Dexamethasone (pharmacology, therapeutic use)
• Dose-Response Relationship, Drug
• Female
• Glucocorticoids (pharmacology, therapeutic use)
• Humans
• Hyperandrogenism (physiopathology)
• Lumbar Vertebrae (diagnostic imaging, physiopathology)
• Steroid 21-Hydroxylase (genetics)
• Tomography, X-Ray Computed
• Young Adult