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BioMed Research International
Volume 2013 (2013), Article ID 250462, 8 pages
http://dx.doi.org/10.1155/2013/250462
Review Article

Glucocorticoid-Induced Osteoporosis in Children with 21-Hydroxylase Deficiency

1Department of Biomedical Sciences and Human Oncology, University of Bari, Piazza G. Cesare 11, 70124 Bari, Italy
2Section of Human Anatomy and Histology, Department of SMB-NOS, University of Bari, 70124 Bari, Italy

Received 20 July 2012; Accepted 4 October 2012

Academic Editor: Leila Zanatta

Copyright © 2013 Annamaria Ventura et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

21-Hydroxylase deficiency (21-OHD) is the most common cause of congenital adrenal hyperplasia (CAH), resulting from deletions or mutations of the P450 21-hydroxylase gene (CYP21A2). Children with 21-OHD need chronic glucocorticoid (cGC) therapy, both to replace congenital deficit in cortisol synthesis and to reduce androgen secretion by adrenal cortex. GC-induced osteoporosis (GIO) is the most common form of secondary osteoporosis that results in an early, transient increase in bone resorption accompanied by a decrease in bone formation, maintained for the duration of GC therapy. Despite the conflicting results in the literature about the bone status on GC-treated patients with 21-OHD, many reports consider these subjects to be at risk for osteoporosis and fractures. In bone cells, at the molecular level, GCs regulate various functions including osteoblastogenesis, osteoclastogenesis, and the apoptosis of osteoblasts and osteocytes. In this paper, we focus on the physiology and biosynthesis of endogenous steroid hormones as well as on the effects of GCs on bone cells, highlighting the pathogenetic mechanism of GIO in children with 21-OHD.