Bone Health Should Be an Important Concern in the Care of Patients Affected by 21 Hydroxylase Deficiency
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Review Article Bone Health Should Be an Important Concern in the Care of Patients Affected by 21 Hydroxylase Deficiency Anne Bachelot,1, 2 Zeina Chakhtoura,1, 2 Dinane Samara-Boustani,3 J´erome Dulon,1, 2 Philippe Touraine,1, 2 and Michel Polak3 1 AP-HP,
Department of Endocrinology and Reproductive Medicine, Groupe Hospitalier Piti´e-Salp´etri`ere, 47-83 boulevard de l’Hˆopital, 75013 Paris Cedex 13, France 2 Centre de R´ ef´erence des Maladies Endocriniennes Rares de la Croissance, Groupe Hospitalier Piti´e-Salp´etri`ere, 47-83 boulevard de l’Hˆopital, 75013 Paris Cedex 13, France 3 AP-HP, Department of Pediatric Endocrinology and Gynecology, Hˆ opital Necker-Enfants Malades, 149 rue de S`evres, 75015 Paris, France Correspondence should be addressed to Philippe Touraine, [email protected] and Michel Polak, [email protected] Received 6 April 2010; Accepted 8 July 2010 Academic Editor: John Fuqua Copyright © 2010 Anne Bachelot 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. Osteoporosis has been an understandable concern for children and adult patients with congenital adrenal hyperplasia (CAH) who may receive or have received supraphysiological doses of glucocorticoids. Some previous reports on bone mineral density (BMD) in adult CAH patients showed no significant differences in BMD between patients with CAH and controls, but others have found lower BMD in CAH patients. In reports documenting the BMD reduction, this outcome has been attributed to an accumulated effect of prolonged exposure to excess glucocorticoids during infancy and childhood. We recently conducted a trial to establish the role of the total cumulative glucocorticoid dose on BMD. We established for the first time that there was a negative relationship between total cumulative glucocorticoid dose and lumbar and femoral BMD. Women might benefit from the preserving effect of estrogens compared to men. BMI (Body Mass Index) also appeared to protect patients from bone loss. In light of this, physicians should bear in mind the potential consequences of glucocorticoids on bone and therefore adjust the treatment and improve clinical and biological surveillance from infancy. Furthermore, preventive measures against corticosteroid-induced osteoporosis should be discussed right from the beginning of glucocorticoid therapy.
1. Introduction Congenital adrenal hyperplasia (CAH, MIM 201910) describes a group of inherited autosomal recessive disorders characterized by enzyme defects in the steroidogenic pathways that lead to the biosynthesis of cortisol, aldosterone, and androgens. Many different mutations of the CYP21 gene have been identified causing varying degrees of impairment of 21-hydroxylase activity that result in a spectrum of disease expression [1]. Most patients are compound heterozygotes, and the clinical phenotype is generally related to the less severely mutated
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