Document Detail

Management of congenital adrenal hyperplasia during pregnancy.
MedLine Citation:
PMID:  15251501     Owner:  NLM     Status:  PubMed-not-MEDLINE    
OBJECTIVE: To provide an overview of the congenital adrenal hyperplasias (CAHs) and their management during pregnancy.
METHODS: Pathways of steroid biosynthesis and inherited deficiencies of required enzymes are reviewed, and applications to prenatal diagnosis and treatment of affected fetuses are discussed.
RESULTS: The CAHs are a group of inherited enzymatic defects of adrenal steroid biosynthesis. During pregnancy, maternal problems are confined to women with 21-hydroxylase deficiency, 11b-hydroxylase deficiency, and 3b-hydroxysteroid dehydrogenase deficiency because other adrenal enzyme deficiencies are incompatible with fertility. The interposition of the placenta on the hypothalamic-pituitary-adrenal axis has a major effect on clinical evaluation of CAH during pregnancy. Women with severe forms of CAH have decreased fertility rates because of oligo-ovulation, and successful conception requires a combination of good therapeutic compliance, careful endocrine monitoring, and often induction of ovulation. 21-Hydroxylase deficiency in the fetus can now be diagnosed accurately prenatally by endocrine testing and molecular genetic techniques. Prenatal diagnosis of 11b-hydroxylase deficiency in the fetus by endocrine testing is not as sensitive. Prevention of masculinization of affected female fetuses by corticosteroid suppression has been attempted in both 21-hydroxylase deficiency and 11b-hydroxylase deficiency CAH, with variable degrees of success. To date, no reports have been published of prenatal diagnosis or treatment of affected female fetuses with 3b-hydroxysteroid dehydrogenase deficiency CAH.
CONCLUSION: Endocrine and genetic studies of CAH during pregnancy have improved the diagnosis and management.
P R Garner
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists     Volume:  2     ISSN:  1530-891X     ISO Abbreviation:  Endocr Pract     Publication Date:    1996 Nov-Dec
Date Detail:
Created Date:  2004-07-14     Completed Date:  2005-02-11     Revised Date:  2014-07-28    
Medline Journal Info:
Nlm Unique ID:  9607439     Medline TA:  Endocr Pract     Country:  United States    
Other Details:
Languages:  eng     Pagination:  397-405     Citation Subset:  -    
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