Literature DB >> 16926248

Reduced bone mineral density and increased bone metabolism rate in young adult patients with 21-hydroxylase deficiency.

Mariateresa Sciannamblo1, Gianni Russo, Debora Cuccato, Giuseppe Chiumello, Stefano Mora.   

Abstract

CONTEXT: Patients with congenital adrenal hyperplasia (CAH) receive glucocorticoids as replacement therapy. Glucocorticoid therapy is the most frequent cause of drug-induced osteoporosis.
OBJECTIVE: The objective of the study was to evaluate bone mineral density (BMD) and bone metabolism in CAH patients.
DESIGN: This was a cross-sectional observational study.
SETTING: The study was conducted at a referral center for pediatric endocrinology. PATIENTS AND OTHER PARTICIPANTS: Thirty young patients with the classical form of CAH (aged 16.4-29.7 yr) treated with glucocorticoid from diagnosis (duration of treatment 16.4-29.5 yr) and 138 healthy controls (aged 16.0-30.0 yr) were enrolled. MAIN OUTCOME MEASURES: BMD was measured in the lumbar spine and whole body by dual-energy x-ray absorptiometry. Bone formation and resorption rates were estimated by serum measurements of bone-specific alkaline phosphatase and C-terminal telopeptide of type I collagen, respectively.
RESULTS: CAH patients were shorter than controls (women -6.8 and men -13.3 cm). Therefore, several methods were used to account for the effect of this difference on bone measurements. Whole-body BMD measurements were significantly lower, compared with controls (P < 0.03), after controlling for height (on average -2.5% in females and -9.3% in male patients). No differences were found in lumbar spine measurements. Bone-specific alkaline phosphatase and C-terminal telopeptide of type I collagen serum concentrations were higher in CAH patients than control subjects (P < 0.04). BMD measurements and bone metabolism markers did not correlate with the actual glucocorticoid dose or mean dose over the previous 7 yr.
CONCLUSIONS: Young adult patients with the classical form of CAH have decreased bone density values, compared with healthy controls. This may put them at risk of developing osteoporosis early in life.

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Year:  2006        PMID: 16926248     DOI: 10.1210/jc.2005-2823

Source DB:  PubMed          Journal:  J Clin Endocrinol Metab        ISSN: 0021-972X            Impact factor:   5.958


  21 in total

1.  Cortical bone mineral density in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Authors:  Diala El-Maouche; Suzanne Collier; Mala Prasad; James C Reynolds; Deborah P Merke
Journal:  Clin Endocrinol (Oxf)       Date:  2014-06-28       Impact factor: 3.478

Review 2.  Clinical outcomes in the management of congenital adrenal hyperplasia.

Authors:  Henrik Falhammar; Marja Thorén
Journal:  Endocrine       Date:  2012-01-07       Impact factor: 3.633

Review 3.  Nonclassic congenital adrenal hyperplasia due to 21-hydroxylase deficiency: clinical presentation, diagnosis, treatment, and outcome.

Authors:  Henrik Falhammar; Anna Nordenström
Journal:  Endocrine       Date:  2015-06-17       Impact factor: 3.633

4.  Management of the adult with congenital adrenal hyperplasia.

Authors:  Richard J Auchus
Journal:  Int J Pediatr Endocrinol       Date:  2010-05-30

5.  Growth and reproductive outcomes in congenital adrenal hyperplasia.

Authors:  Todd D Nebesio; Erica A Eugster
Journal:  Int J Pediatr Endocrinol       Date:  2010-02-01

6.  An Evidence-Based Model of Multidisciplinary Care for Patients and Families Affected by Classical Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency.

Authors:  Traci L Schaeffer; Jeanie B Tryggestad; Ashwini Mallappa; Adam E Hanna; Sowmya Krishnan; Steven D Chernausek; Laura J Chalmers; William G Reiner; Brad P Kropp; Amy B Wisniewski
Journal:  Int J Pediatr Endocrinol       Date:  2010-03-18

7.  Bone health should be an important concern in the care of patients affected by 21 hydroxylase deficiency.

Authors:  Anne Bachelot; Zeina Chakhtoura; Dinane Samara-Boustani; Jérome Dulon; Philippe Touraine; Michel Polak
Journal:  Int J Pediatr Endocrinol       Date:  2010-09-28

8.  Correlation of Bone Mineral Parameters with Anthropometric Measurements and the Effect of Glucocorticoids on Bone Mineral Parameters in Congenital Adrenal Hyperplasia.

Authors:  Ramaswamy Ganesh; Natarajan Suresh; Lalitha Janakiraman; Karnam Ravikumar
Journal:  Indian J Pediatr       Date:  2015-07-31       Impact factor: 1.967

9.  Approach to the adult with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Authors:  Deborah P Merke
Journal:  J Clin Endocrinol Metab       Date:  2008-03       Impact factor: 5.958

Review 10.  Approach to the patient: the adult with congenital adrenal hyperplasia.

Authors:  Richard J Auchus; Wiebke Arlt
Journal:  J Clin Endocrinol Metab       Date:  2013-07       Impact factor: 5.958

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