Experience in optimizing fertility outcomes in men with congenital adrenal hyperplasia due to 21 hydroxylase deficiency

Summary Objective Men with congenital adrenal hyperplasia (CAH) have impaired fertility. We aimed to assess fertility outcomes and the importance of hypogonadotropic hypogonadism, testicular failure and the presence of testicular adrenal rest tumours (TART). Design Retrospective analysis of men atte...

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Bibliographic Details
Published in:Clinical endocrinology (Oxford) 2016-06, Vol.84 (6), p.830-836
Main Authors: King, Thomas F.J., Lee, Marilyn Cheng, Williamson, Elizabeth E.J., Conway, Gerard S.
Format: Article
Language:English
Subjects:
Adolescent
Adrenal Hyperplasia, Congenital - complications
Adrenal Rest Tumor - diagnostic imaging
Adult
Androstenedione
Cryopreservation
Fertility
Follicle Stimulating Hormone - analysis
Follicle-stimulating hormone
Humans
Hyperplasia
Hypogonadism
In vitro fertilization
Infertility, Male - diagnosis
Infertility, Male - etiology
Infertility, Male - therapy
Luteinizing hormone
Luteinizing Hormone - analysis
Male
Middle Aged
Reproductive Techniques, Assisted
Reproductive technologies
Reproductive technology
Retrospective Studies
Semen Analysis
Sperm
Testes
Testicular Neoplasms - diagnostic imaging
Treatment Outcome
Ultrasonic imaging
Ultrasonography
Ultrasound
Young Adult
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Summary:Summary Objective Men with congenital adrenal hyperplasia (CAH) have impaired fertility. We aimed to assess fertility outcomes and the importance of hypogonadotropic hypogonadism, testicular failure and the presence of testicular adrenal rest tumours (TART). Design Retrospective analysis of men attending an adult CAH clinic in a tertiary centre. Patients Fifty men with CAH due to 21 hydroxylase deficiency were identified of whom 35 were salt wasting and 15 were non‐salt‐wasting. Measurements Review of fertility history and parameters including luteinizing hormone (LH), follicle‐stimulating hormone (FSH), androstenedione, 17‐hydroxyprogesterone (17‐OHP), semen analysis and the presence of testicular adrenal rest tissue (TART) on ultrasound. Results TART were detected by ultrasound in 21 (47%), and their presence was associated with an elevated FSH (P = 0·01). Severe oligospermia was present in 11 of 23 (48%), and this was associated with an elevated FSH (P = 0·02), suppressed LH (P < 0·01) and TART (P = 0·03) when compared to those with a sperm count >5 × 106 per ml. Of those that desired fertility, 10 of 17 (59%) required treatment intensification and four underwent in vitro fertilization. Intensification resulted in a rise in median LH (0·6–4·3 IU/l; P = 0·01). Live birth rate was 15 of 17 (88%) with a median (range) time to conception of 8 (0–38) months. Conclusions Suppressed LH is a marker for subfertility and is often reversible. Testicular failure is closely associated with TART formation. If TART are detected, sperm cryopreservation should be offered given the risk of progression to irreversible testicular failure. Male fertility in CAH can be improved by intensified treatment and assisted reproductive technology.
ISSN:0300-0664
1365-2265
DOI:10.1111/cen.13001