Reassessing fecundity in women with classical congenital adrenal hyperplasia (CAH): normal pregnancy rate but reduced fertility rate
Abstract
Summary: Objective Fertility in women with classical congenital adrenal hyperplasia (CAH) has been reported low; however, the true pregnancy rate for women trying to conceive with this condition is unknown. Our aim was to calculate pregnancy rate for women with CAH calculated as a proportion of those who had attempted conception. Fertility expressed as live birth rate is also calculated. Patients One hundred and six women with classical CAH followed in a multidisciplinary service [81 salt‐losing (SL) and 25 nonsalt‐losing (NSL) form]. Results Twenty‐five (23·6%) women with CAH women considered motherhood, 23 had actively tried conception of whom 21 (91·3%) achieved 34 pregnancies. Pregnancy rate is no different from that in the normal population (95%). Pregnancy rates were similar in the SL (88·9%) and NSL (92·9%) subgroups but those with NSL‐CAH were more likely to seek motherhood than those with SL‐CAH (16/25 vs. 9/81). Optimized glucocorticoid and mineralocorticoid regimes during fertility monitoring resulted spontaneous conception in nearly all recent cases. Fertility rate was 0·25 live births per woman compared with 1·8 in the UK population ( P < 0·001). Conclusion We report a normal pregnancy rate (91·3%) for women with classical CAH, similar in SL and NSL subgroups. Fertility rate, however, remains much lower than in general population.
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Suppression of follicular phase progesterone enhances the likelihood of ovulation and subsequent conception.
“suppression of follicular phase progesterone enhances the likelihood of ovulation and subsequent conception.110,111”
From Congenital adrenal hyperplasia (2017) · cites this paper as [110]
Several studies show that only a minority of women with classic CAH try to conceive.
“Several studies have shown that, for a variety of reasons, only a minority of women with classic CAH try to conceive (225, 257).”
From Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline (2018) · cites this paper as [225]
Women with classic CAH who wish to conceive can achieve nearly normal pregnancy rates with adequate suppression of follicular-phase progesterone by optimizing glucocorticoid and mineralocorticoid treatments.
“Those who wish to conceive can achieve nearly normal pregnancy rates with adequate suppression of follicular-phase progesterone (,0.6 ng/ mL = 2 nmol/L, Table 5) (225) by optimizing GC and MC treatments.”
From Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline (2018) · cites this paper as [225]
Caesarean section is the most common delivery method in CAH due to previous vaginal surgery and cephalopelvic disproportion; vaginal delivery has been reported in 16% to 42% of women, nearly all with a non-salt-wasting phenotype.
“Caesarean section is the most common method of delivery due to the high prevalence of previous vaginal surgery and cephalopelvic disproportion, although vaginal delivery has been reported in 16% to 42% of women, nearly all of whom had a non-salt-wasting phenotype (225, 257).”
From Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline (2018) · cites this paper as [225]
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- Authors
- Casteràs, A., De Silva, P., Rumsby, G., & Conway, G. S.
- Affiliations
- University College Hospital (GB)
- Corresponding author
- Conway, Gerard S.
- Venue
- Clinical Endocrinology
- Publisher
- Wiley
- Published
- 2009-05-13
- Volume
- 70
- Issue
- 6
- Pages
- 833-837
- ISSN
- 0300-0664 (ISSN-L)
- 1365-2265
- OpenAlex
- W2028550929
- Cited by
- 190
- References
- 25
- Citations by year
- 2026: 8 · 2025: 12 · 2024: 7 · 2023: 13 · 2022: 17 · 2021: 14 · 2020: 12 · 2019: 9 · 2018: 9 · 2017: 8 · 2016: 11 · 2015: 10 · 2014: 8 · 2013: 18 · 2012: 11
- FWCI
- 4.56
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Clinical Endocrinology (publishedVersion)
- PubMed (publishedVersion)
- UCL Discovery (University College London) (submittedVersion)
- Funders
- National Institute for Health and Care Research
- University College London Hospitals NHS Foundation Trust
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Health Sciences › Medicine › Reproductive Medicine › Ovarian function and disorders
- Keywords
- classical congenital adrenal hyperplasia, female fertility, pregnancy rate, live birth rate, glucocorticoid therapy, mineralocorticoid therapy, fertility monitoring, spontaneous conception
- MeSH terms
- Adolescent, Adrenal Hyperplasia, Congenital, Adult, Birth Rate, Female, Fertility, Follow-Up Studies, Humans, Middle Aged, Pregnancy, Cohort Studies, Pregnancy Rate, Young Adult
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