Gonadal function in adult male patients with congenital adrenal hyperplasia
| Autor: | M Engels, K Gehrmann, H Falhammar, E A Webb, A Nordenström, F C Sweep, P N Span, A E van Herwaarden, J Rohayem, A Richter-Unruh, C Bouvattier, B Köhler, B B Kortmann, W Arlt, N Roeleveld, N Reisch, N M M L Stikkelbroeck, H L Claahsen-van der Grinten, Peggy Cohen-Kettenis, Annelou de Vries, Claudia Wiesemann, Jolanta Slowikowska-Hilczer, Aude Brac de la Perriere, Charles Sultan, Francoise Paris, Ute Thyen, Catherine Pienkowski, Maria Szarras-Czapnik |
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| Rok vydání: | 2018 |
| Předmět: |
Male
Endocrinology Diabetes and Metabolism 0302 clinical medicine Endocrinology Hydroxyprogesterones Odds Ratio Prevalence Medicine Testosterone Women's cancers Radboud Institute for Molecular Life Sciences [Radboudumc 17] 030219 obstetrics & reproductive medicine Sperm Count medicine.diagnostic_test Vascular damage Radboud Institute for Molecular Life Sciences [Radboudumc 16] General Medicine Middle Aged Women's cancers Radboud Institute for Health Sciences [Radboudumc 17] Europe Reconstructive and regenerative medicine Radboud Institute for Molecular Life Sciences [Radboudumc 10] Sperm Motility Gonadotropin Adult medicine.medical_specialty Adolescent medicine.drug_class 030209 endocrinology & metabolism Context (language use) Semen analysis Young Adult 03 medical and health sciences All institutes and research themes of the Radboud University Medical Center Testicular Neoplasms Internal medicine Adrenal Rest Tumor Humans Congenital adrenal hyperplasia Androstenedione Aged Adrenal Hyperplasia Congenital business.industry Hypogonadism Oligospermia Odds ratio medicine.disease Reconstructive and regenerative medicine Radboud Institute for Health Sciences [Radboudumc 10] Semen Analysis Cross-Sectional Studies Testicular adrenal rest tumor business Gonadotropins |
| Zdroj: | European Journal of Endocrinology, 178, 285-294 European Journal of Endocrinology, 178, 3, pp. 285-294 |
| ISSN: | 0804-4643 |
| Popis: | Context Current knowledge on gonadal function in congenital adrenal hyperplasia (CAH) is mostly limited to single-center/country studies enrolling small patient numbers. Overall data indicate that gonadal function can be compromised in men with CAH. Objective To determine gonadal function in men with CAH within the European ‘dsd-LIFE’ cohort. Design Cross-sectional clinical outcome study, including retrospective data from medical records. Methods Fourteen academic hospitals included 121 men with CAH aged 16–68 years. Main outcome measures were serum hormone concentrations, semen parameters and imaging data of the testes. Results At the time of assessment, 14/69 patients had a serum testosterone concentration below the reference range; 7 of those were hypogonadotropic, 6 normogonadotropic and 1 hypergonadotropic. In contrast, among the patients with normal serum testosterone (55/69), 4 were hypogonadotropic, 44 normogonadotropic and 7 hypergonadotropic. The association of decreased testosterone with reduced gonadotropin concentrations (odds ratio (OR) = 12.8 (2.9–57.3)) was weaker than the association between serum androstenedione/testosterone ratio ≥1 and reduced gonadotropin concentrations (OR = 39.3 (2.1–732.4)). Evaluation of sperm quality revealed decreased sperm concentrations (15/39), motility (13/37) and abnormal morphology (4/28). Testicular adrenal rest tumor (TART)s were present in 39/80 patients, with a higher prevalence in patients with the most severe genotype (14/18) and in patients with increased current 17-hydroxyprogesterone 20/35) or androstenedione (12/18) serum concentrations. Forty-three children were fathered by 26/113 patients. Conclusions Men with CAH have a high risk of developing hypothalamic-pituitary-gonadal disturbances and spermatogenic abnormalities. Regular assessment of endocrine gonadal function and imaging for TART development are recommended, in addition to measures for fertility protection. |
| Databáze: | OpenAIRE |
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