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Disorders of Adrenal Steroidogenesis: Impact on Gonadal Function and Sex Development.
- Source :
-
Pediatric endocrinology reviews : PER [Pediatr Endocrinol Rev] 2016 Dec; Vol. 14 (2), pp. 109-128. - Publication Year :
- 2016
-
Abstract
- A defect in adrenal steroidogenesis may cause a disorder of sex development (DSD). Importantly, DSD of adrenal origin is not restricted to a genital phenotype but is in most cases accompanied by mild to severe impairment in glucocorticoid and/or mineralocorticoid synthesis. If a patient is suspected of DSD of adrenal origin evaluation of glucocorticoid and mineralocorticoid metabolism is therefore essential to provide adequate medical care in the case of a severe and potentially life-threatening insufficiency. The adrenal steroidogenic defects causing DSD, their clinical features and diagnostic work-up are discussed. In this review we provide an overview of defects in the adrenal steroidogenesis and its impact on gonadal function and sex development.<br /> (Copyright© of YS Medical Media ltd.)
Details
- Language :
- English
- ISSN :
- 1565-4753
- Volume :
- 14
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Pediatric endocrinology reviews : PER
- Publication Type :
- Academic Journal
- Accession number :
- 28508605
- Full Text :
- https://doi.org/10.17458/PER.2016.MEC.DisordersofAdrenal