Document Detail

Classical forms of congenital adrenal hyperplasia due to 21-hydroxylase deficiency in adults.
MedLine Citation:
PMID:  18204267     Owner:  NLM     Status:  MEDLINE    
During childhood, the main aims of the medical treatment of congenital adrenal hyperplasia (CAH) secondary to 21-hydroxylase are to prevent salt loss and virilization and to attain normal stature and normal puberty. As such, there is a narrow therapeutic window through which the intended results can be achieved. In adulthood, the clinical management has received little attention, but recent studies have shown the relevance of long-term follow-up of these patients. The aims here are to review the multiple clinical, hormonal and metabolic abnormalities that could be found in adult CAH patients as such a decrease in bone mineral density, overweight and disturbed reproductive functions. In women with classic CAH, a low fertility rate is reported, and is probably the consequence of multiple factors including neuroendocrine and hormonal factors, feminizing surgery, and psychological factors. Men with CAH may present hypogonadism either through the effect of adrenal rests or from suppression of gonadotropins resulting in infertility. Therefore a multidisciplinary team with knowledge of CAH should carefully follow up these patients, from childhood through to adulthood, to avoid these complications and to ensure treatment compliance and tight control of the adrenal androgens.
Anne Bachelot; Zeina Chakthoura; Agnès Rouxel; Jérome Dulon; Philippe Touraine
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review     Date:  2008-01-21
Journal Detail:
Title:  Hormone research     Volume:  69     ISSN:  1423-0046     ISO Abbreviation:  Horm. Res.     Publication Date:  2008  
Date Detail:
Created Date:  2008-04-17     Completed Date:  2008-05-20     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0366126     Medline TA:  Horm Res     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  203-11     Citation Subset:  IM    
Copyright Information:
(c) 2008 S. Karger AG, Basel
Department of Endocrinology and Reproductive Medicine, Groupe Hospitalier Pitié-Salpétrière, Paris, France.
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MeSH Terms
Adrenal Hyperplasia, Congenital / complications,  etiology*,  therapy
Infertility / etiology
Metabolism, Inborn Errors / complications*
Obesity / etiology
Osteoporosis / etiology
Steroid 21-Hydroxylase / metabolism*
Reg. No./Substance:
EC 21-Hydroxylase

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