Classical forms of congenital adrenal hyperplasia due to 21-hydroxylase deficiency in adults

Hormone Research
Anne BachelotPhilippe Touraine

Abstract

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...Continue Reading

References

Oct 26, 1978·The New England Journal of Medicine·L S LevineB Dupont
Nov 1, 1991·The Journal of Clinical Endocrinology and Metabolism·J Blumberg-TickG Schaison
Dec 27, 1990·The New England Journal of Medicine·G B Cutler, L Laue
Jan 22, 1987·The New England Journal of Medicine·R M MulaikalJ A Rock
Mar 1, 1997·Clinical Endocrinology·L D PremawardhanaM F Scanlon
Jun 5, 1998·Archives of Disease in Childhood·R E CorneanC G Brook
Sep 28, 1998·The New England Journal of Medicine·G RussoG Chiumello
Apr 21, 1999·The Journal of Urology·N K AlizaiN Johnson
Feb 7, 2001·The Journal of Clinical Endocrinology and Metabolism·H BruiningS W Lamberts
May 10, 2001·Endocrinology and Metabolism Clinics of North America·B L Therrell
May 10, 2001·Endocrinology and Metabolism Clinics of North America·J C Lo, M M Grumbach
Oct 31, 2001·The American Journal of Surgical Pathology·H A Al-AhmadieR H Young
May 8, 2002·The Journal of Clinical Endocrinology and Metabolism·Evangelia CharmandariDeborah P Merke
Mar 12, 2003·The Journal of Clinical Endocrinology and Metabolism·Nike M M L StikkelbroeckBarto J Otten
Oct 31, 2003·Journal of Bone and Mineral Metabolism·Patrícia Oliveira de Almeida FreireGil Guerra
Mar 31, 2004·Journal of Pediatric and Adolescent Gynecology·Jill HuppertPaula J A Hillard
Jun 21, 2005·Lancet·Deborah P Merke, Stefan R Bornstein
Jul 26, 2005·Growth Hormone & IGF Research : Official Journal of the Growth Hormone Research Society and the International IGF Research Society·Mary Pat GallagherSharon E Oberfield
Nov 10, 2005·The Journal of Clinical Endocrinology and Metabolism·Jeremy A KingClaude J Migeon
Jan 13, 2006·Clinical Endocrinology·Cara Megan OgilvieGerard S Conway
Feb 25, 2006·European Journal of Endocrinology·Cara Megan OgilvieGerard S Conway
Aug 24, 2006·The Journal of Clinical Endocrinology and Metabolism·Mariateresa SciannambloStefano Mora
Sep 28, 2006·The Journal of Clinical Endocrinology and Metabolism·Thomas M K VölklHelmuth G Dörr
Oct 13, 2006·The Journal of Clinical Endocrinology and Metabolism·Henrik FalhammarMarja Thorén

❮ Previous
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Citations

Jan 29, 2011·International Journal of Pediatric Endocrinology·Richard J AuchusAlan E Zuckerman
Jul 31, 2010·International Journal of Pediatric Endocrinology·Selma Feldman Witchel, Ricardo Azziz
Dec 2, 2009·Journal of Pediatric Nursing·Carol Van Ryzin
Feb 4, 2012·International Journal of Pediatric Endocrinology·Sarita A SanchesHedi L Claahsen-van der Grinten
Dec 18, 2013·Nature Reviews. Endocrinology·Thang S HanRichard J Ross
Dec 4, 2013·Critical Reviews in Clinical Laboratory Sciences·Chloe Miu MakChing-Wan Lam
Jan 25, 2012·The American Journal of the Medical Sciences·Mouna Feki MnifMohamed Abid
Oct 23, 2013·Seminars in Diagnostic Pathology·Anne Marie McNicol
May 24, 2011·Journal of Pediatric and Adolescent Gynecology·Selma Feldman Witchel, Ricardo Azziz
Oct 11, 2008·Dermatologic Therapy·Cem Demirci, Selma Feldman Witchel
Mar 1, 2009·Dermato-endocrinology·Cleo Dessinioti, Andreas Katsambas
Feb 1, 2020·Journal of Pediatric Endocrinology & Metabolism : JPEM·Noha MusaLubna Fawaz
Feb 2, 2013·Diagnostic Molecular Pathology : the American Journal of Surgical Pathology, Part B·Paola ConcolinoEttore Capoluongo
Aug 17, 2021·Frontiers in Endocrinology·Cinzia CarrozzaPaola Concolino

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