Literature DB >> 17170529

Long-term outcome of patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Anne Bachelot1, Geneviève Plu-Bureau, Elisabeth Thibaud, Kathleen Laborde, Graziella Pinto, Dinane Samara, Claire Nihoul-Fékété, Frédérique Kuttenn, Michel Polak, Philippe Touraine.   

Abstract

AIMS: Conflicting results exist regarding bone mineral density (BMD), metabolism and reproductive function of adult patients with congenital adrenal hyperplasia (CAH). We evaluated the long-term outcome and the impact of chronic glucocorticoid replacement in these patients.
METHODS: Physical characteristics, serum hormone concentrations, BMD and metabolism were studied in 45 consecutive CAH adult patients.
RESULTS: Among the 36 women, only 14 (39%) had regular menses. Among the 27 women with classical CAH, the mean number of surgical reconstructions of virilized genitalia was 2.1 +/- 0.2. Twenty of them (74%) were sexually active. Three men presented with testicular adrenal rest tumors. Twenty-five patients (55%) had decreased BMD at the femoral neck and/or at the lumbar spine. BMI was correlated with the BMD T-score at the femoral neck (p < 0.001) and at the lumbar spine (p < 0.01). Hydrocortisone dose was negatively correlated with the BMD T-score at the femoral neck (p = 0.04). Subjects with osteopenia had a significantly lower BMI and received higher hydrocortisone dose than those with normal BMD. Overweight was found in 21 patients (47%). There was a significantly positive correlation between HOMA and BMI (p < 0.001), and between HOMA and 17-OHP levels (p = 0.016).
CONCLUSIONS: Adult patients with CAH treated with long-term glucocorticoids are at risk for decreased BMD, increased BMI, and disturbed reproductive function. Copyright 2007 S. Karger AG, Basel.

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Year:  2006        PMID: 17170529     DOI: 10.1159/000098017

Source DB:  PubMed          Journal:  Horm Res        ISSN: 0301-0163


  30 in total

Review 1.  The way toward adulthood for females with nonclassic congenital adrenal hyperplasia.

Authors:  Georgia Ntali; Sokratis Charisis; Christo F Kylafi; Evangelia Vogiatzi; Lina Michala
Journal:  Endocrine       Date:  2021-04-14       Impact factor: 3.633

Review 2.  Clinical perspectives in congenital adrenal hyperplasia due to 11β-hydroxylase deficiency.

Authors:  Krupali Bulsari; Henrik Falhammar
Journal:  Endocrine       Date:  2016-12-07       Impact factor: 3.633

3.  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

4.  Increased large artery intima media thickness in adolescents with either classical or non-classical congenital adrenal hyperplasia.

Authors:  M Wasniewska; A Balsamo; M Valenzise; A Manganaro; G Faggioli; S Bombaci; V Conti; M Ferri; T Aversa; A Cicognani; F De Luca
Journal:  J Endocrinol Invest       Date:  2011-12-21       Impact factor: 4.256

5.  Carotid intima media thickness and other cardiovascular risk factors in children with congenital adrenal hyperplasia.

Authors:  N H Amr; A Y Ahmed; Y A Ibrahim
Journal:  J Endocrinol Invest       Date:  2014-08-12       Impact factor: 4.256

6.  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

Review 7.  [Testicular adrenal rest tumors (TART) in adult men with classic congenital adrenal hyperplasia (CAH)].

Authors:  P Knape; N Reisch; H-G Dörr; M Reincke; S Lenk; M Quinkler
Journal:  Urologe A       Date:  2008-12       Impact factor: 0.639

Review 8.  [Congenital adrenal hyperplasia and growth hormone deficiency. Special care in transition to adulthood].

Authors:  H G Dörr; C Schöfl
Journal:  Internist (Berl)       Date:  2009-10       Impact factor: 0.743

Review 9.  Cardiovascular disease risk in adult women with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Authors:  Mimi S Kim; Deborah P Merke
Journal:  Semin Reprod Med       Date:  2009-06-15       Impact factor: 1.303

Review 10.  Recent advances in diagnosis, treatment, and outcome of congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

Authors:  Felix G Riepe; Wolfgang G Sippell
Journal:  Rev Endocr Metab Disord       Date:  2007-12       Impact factor: 6.514

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