The DOI matches its Crossref record; 22 of the 22 references it cites were found in a registry or an index.
High Prevalence of Reduced Fecundity in Men with Congenital Adrenal Hyperplasia
Abstract
Context: Testicular adrenal rest tumors (TARTs) are regarded to contribute to the high prevalence of subfertility in males with congenital adrenal hyperplasia (CAH). Objectives: Our objectives were to evaluate reduced fecundity and its possible causes in well-controlled adult males with CAH, and to investigate diagnostic tools for improved treatment monitoring with respect to fertility outcomes. Design: In a cross-sectional study at the Department of Endocrinology at the University Hospital München, Germany, 22 adult male CAH patients (15 salt wasting and seven simple virilizing, age 19–48 yr) were clinically assessed according to their hormonal control. We performed testicular ultrasound (22 of 22), magnetic resonance imaging (18 of 22), and a semen analysis (19 of 22) in the participants. Results: All patients had a pathological semen analysis. TART prevalence was 10 of 22 (eight salt wasting, two simple virilizing). Poor therapy control was present in five patients, and all five had TARTs. Of the other 17 well-controlled patients with normal or suppressed adrenal androgens and 17-hydroxyprogesterone levels, five presented with TARTs. There was a significant correlation between sperm concentration and functional testicular volume (r = 0.70; P = 0.002), TART volume (r = −0.70; P = 0.036), as well as inhibin B levels (r = 0.75; P < 0.0001), respectively. In several men, hormonal control parameters suggested hypogonadism, with glucocorticoid overtreatment as a relevant factor for poor semen quality. Conclusions: Poor semen parameters are common in male CAH patients. TARTs, most likely reflecting undertreatment, as well as inhibin B are important indicators of fecundity. On the other hand, long-term glucocorticoid overtreatment also seems to contribute to low semen quality.
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In the citing paper
“In the virilizing forms of CAH, excess adrenal sex steroids can lead to hypogonadotropic hypogonadism 106,107 and increased progesterone can interfere with endometrial implantation.108,109”
From Congenital adrenal hyperplasia (2017) · cites this paper as [106]
In the citing paper
“Low inhibin B levels reflect declining Sertoli cell function and can be used to monitor.106”
From Congenital adrenal hyperplasia (2017) · cites this paper as [106]
In the citing paper
“Fertility in males with CAH is often impaired (226, 237, 248-250).”
From Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline (2018) · cites this paper as [248]
In the citing paper
“TARTs have no malignant features but can lead to obstructive azoospermia and infertility (248).”
From Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline (2018) · cites this paper as [248]
In the citing paper
“The presence of TARTs is a predictor of infertility (226, 248, 253).”
From Congenital Adrenal Hyperplasia Due to Steroid 21-Hydroxylase Deficiency: An Endocrine Society* Clinical Practice Guideline (2018) · cites this paper as [248]
Citations
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- Bals-Pratsch, 1999, World Health Organization (WHO)-Laborhandbuch
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Speiser, P. W., Azziz, R., Baskin, L. S., Ghizzoni, L., Hensle, T. W., Merke, D. P., Meyer-Bahlburg, H. F. L., Miller, W. L., Montori, V. M., Oberfield, S. E., et al. (2010) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2009-2631 · Cited by 1,468
“Fertility may be impaired by suppression of gonadotropin secretion by adrenal steroids if adequate doses of GCs are not given (242).”
Speiser, P. W., Arlt, W., Auchus, R. J., Baskin, L. S., Conway, G. S., Merke, D. P., Meyer-Bahlburg, H. F. L., Miller, W. L., Murad, M. H., Oberfield, S. E., et al. (2018) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2018-01865 · Cited by 1,234
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“Decrease in these steroid hormones signals the adrenals to produce more androgens, leading to virilization of genitalia in females [78] and subfertility in males [79].”
BackgroundCongenital adrenal hyperplasia
El-Maouche, D., Arlt, W., & Merke, D. P. (2017) · The Lancet · doi:10.1016/s0140-6736(17)31431-9 · Cited by 669
Congenital Adrenal Hyperplasia—Current Insights in Pathophysiology, Diagnostics, and Management
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Self-citation
Metadata
- Authors
- Reisch, N., Flade, L., Scherr, M., Rottenkolber, M., Pedrosa Gil, F., Bidlingmaier, M., Wolff, H., Schwarz, H.-P., Quinkler, M., Beuschlein, F., & Reincke, M.
- Affiliations
- Ludwig-Maximilians-Universität München (DE)
- LMU Klinikum (DE)
- Psychosomatic Ambulance and Outpatient Clinic (F.P.G.), University Hospital München, D-80336 München, Germany
- München Klinik (DE)
- Charité – Universitätsmedizin Berlin (DE)
- Endocrinology and Metabolism (N.R., L.F., M.B., F.B., M.Re.), University Hospital München, D-80336 München, Germany
- Corresponding author
- Reisch, Nicole
- Venue
- The Journal of Clinical Endocrinology & Metabolism
- Publisher
- The Endocrine Society
- Published
- 2009-05-01
- Volume
- 94
- Issue
- 5
- Pages
- 1665-1670
- ISSN
- 0021-972X (ISSN-L)
- 1945-7197
- OpenAlex
- W1981718662
- Cited by
- 170
- References
- 22
- Citations by year
- 2026: 6 · 2025: 2 · 2024: 6 · 2023: 5 · 2022: 8 · 2021: 9 · 2020: 9 · 2019: 12 · 2018: 6 · 2017: 12 · 2016: 13 · 2015: 12 · 2014: 11 · 2013: 15 · 2012: 15
- FWCI
- 5.72
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › 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
- congenital adrenal hyperplasia, male subfertility, testicular adrenal rest tumor, semen quality, hypogonadism, inhibin B, 17-hydroxyprogesterone, salt wasting, simple virilizing CAH, testicular volume
- MeSH terms
- Adrenal Hyperplasia, Congenital, Adrenal Rest Tumor, Adult, Fertility, Follicle Stimulating Hormone, Hormones, Humans, Infertility, Male, Inhibins, Leydig Cells, Luteinizing Hormone, Male, Middle Aged, Saliva, Semen, Sperm Count, Spermatogenesis, Testis, Testosterone, Ultrasonography, Cohort Studies, Treatment Outcome, Young Adult
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