Fertility in Patients with Congenital Adrenal Hyperplasia
- 1 January 2006
- journal article
- review article
- Published by Walter de Gruyter GmbH in Journal of Pediatric Endocrinology and Metabolism
- Vol. 19 (5), 677-85
- https://doi.org/10.1515/jpem.2006.19.5.677
Abstract
Congenital adrenal hyperplasia (CAH) is generally regarded as a paediatric endocrine disease, but nowadays nearly all patients reach adulthood as a result of improved diagnosis and treatment. It is now increasingly recognised that treatment goals shift during life: one of the major treatment goals in childhood and puberty, i.e. normal growth and development, is no longer relevant after childhood, whereas other aspects, such as fertility and side effects of long-term glucocorticoid treatment, become more important in adulthood. This paper focuses on fertility in male and female adult patients with CAH. In males with CAH the fertility rate is reduced compared with the normal population, the most frequent cause being testicular adrenal rest tumours. Development and growth of these tumours is assumed to be ACTH dependent and undertreatment may play an important role. If intensifying glucocorticoid treatment does not lead to tumour decrease, surgical intervention may be considered, but the effect on fertility is not yet known. In females with CAH the degree of fertility depends on the phenotype of the CAH. Most fertility problems are seen in the classic salt-wasting type. Age of menarche and regularity of the menstrual cycle depends on the degree of adrenal suppression. Not only adrenal androgens have to be normalised but also the levels of adrenal progestins (progesterone and 17-OH-progesterone) that interfere with normal ovulatory cycles. The regularity of menstrual cycles can be considered as an important measure of therapeutic control in adolescent females with CAH and therefore as a therapeutic goal from (peri)pubertal years on. Other factors that contribute to impaired fertility in females with CAH are ovarian hyperandrogenism (polycystic ovary syndrome), ovarian adrenal rest tumours, genital surgery and psychological factors. Subfertility in CAH can have its origin already in the peripubertal years and is therefore of interest to the paediatric endocrinologist.Keywords
This publication has 54 references indexed in Scilit:
- Ovarian Adrenal Rest Tissue in Congenital Adrenal Hyperplasia - A Patient ReportJournal of Pediatric Endocrinology and Metabolism, 2006
- Asymptomatic Testicular Adrenal Rest Tumours in Adolescent and Adult Males with Congenital Adrenal Hyperplasia: Basal and Follow-up Investigation After 2.6 YearsJournal of Pediatric Endocrinology and Metabolism, 2004
- Genital sensation after feminizing genitoplasty for congenital adrenal hyperplasia: a pilot studyBJU International, 2003
- Consensus Statement on 21-Hydroxylase Deficiency from The European Society for Paediatric Endocrinology and The Lawson Wilkins Pediatric Endocrine SocietyHormone Research in Paediatrics, 2002
- High Prevalence of Testicular Adrenal Rest Tumors, Impaired Spermatogenesis, and Leydig Cell Failure in Adolescent and Adult Males with Congenital Adrenal HyperplasiaJournal of Clinical Endocrinology & Metabolism, 2001
- Long Term Outcome in Adult Males with Classic Congenital Adrenal HyperplasiaJournal of Clinical Endocrinology & Metabolism, 2001
- Fertility and Body Composition after Laparoscopic Bilateral Adrenalectomy in a 30-Year-Old Female with Congenital Adrenal HyperplasiaJournal of Clinical Endocrinology & Metabolism, 2001
- Congenital Adrenal Hyperplasia due to 21-Hydroxylase DeficiencyEndocrine Reviews, 2000
- Testis Sparing Surgery for Steroid Unresponsive Testicular Tumors of the Adrenogenital SyndromeJournal of Urology, 1997
- Subnormal androgen and elevated progesterone levels in women treated for congenital virilizing 21-hydroxylase deficiencyJournal of Clinical Endocrinology & Metabolism, 1993