{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T22:05:59Z","timestamp":1785535559951,"version":"3.56.0"},"reference-count":0,"publisher":"Oxford University Press (OUP)","issue":"4","content-domain":{"domain":["eje.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:sec>\n<jats:title>Objective<\/jats:title>\n<jats:p>To study the impact of the quality of therapeutic control on fertility and on the prevalence of testicular adrenal rest tumours (TART) in young males with congenital adrenal hyperplasia (CAH).<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Design<\/jats:title>\n<jats:p>Combined cross-sectional and retrospective clinical study.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>Twenty-nine patients and age-matched controls underwent clinical investigation, including semen analysis, testicular and adrenal ultrasound imaging, and serum and hair steroid analysis. The quality of therapeutic control was categorized as \u2018poor\u2019, \u2018moderate\u2019 or \u2018medium\u2019. Evaluation of current control was based on concentrations of 17-hydroxy-progesterone and androstenedione in serum and 3 cm hair; previous control was categorized based on serum 17-hydroxy-progesterone concentrations during childhood and puberty, anthropometric and puberty data, bone age data and adrenal sizes.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>Semen quality was similar in males with CAH and controls (<jats:italic>P<\/jats:italic>\u2009\u2009=\u20090.066), however patients with \u2018poor\u2019 past control and large TART, or with \u2018poor\u2019 current CAH control had low sperm counts. Follicle-stimulating hormone was decreased, if current CAH control was \u2018poor\u2019 (1.8 \u00b1 0.9 U\/L; \u2018good\u2019: 3.9 \u00b1 2.2 U\/L); <jats:italic>P<\/jats:italic>\u2009\u2009=\u20090.015); luteinizing hormone was decreased if it was \u2018poor\u2019 (1.8 \u00b1 0.9 U\/L; <jats:italic>P<\/jats:italic>\u2009\u2009=\u20090.041) or \u2018moderate\u2019 (1.9 \u00b1 0.6 U\/L; \u2018good\u2019: 3.0 \u00b1 1.3 U\/L; <jats:italic>P<\/jats:italic>\u2009\u2009=\u20090.025). None of the males with \u2018good\u2019 past CAH control, 50% of those with \u2018moderate\u2019 past control and 80% with \u2018poor past control had bilateral TART. The prevalence of TART in males with severe (class null or A) <jats:italic>CYP21A2<\/jats:italic> mutations was 53% and 25% and 0% in those with milder class B and C mutations, respectively.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>TART development is favoured by inadequate long-term hormonal control in CAH. Reduced semen quality may be associated with large TART. Gonadotropin suppression by adrenal androgen excess during the latest spermatogenic cycle may contribute to impairment of spermatogenesis.<\/jats:p>\n<\/jats:sec>","DOI":"10.1530\/eje-20-1154","type":"journal-article","created":{"date-parts":[[2021,2,1]],"date-time":"2021-02-01T19:31:43Z","timestamp":1612207903000},"page":"487-501","update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-7","source":"Crossref","is-referenced-by-count":22,"title":["Semen quality and testicular adrenal rest tumour development in 46,XY congenital adrenal hyperplasia: the importance of optimal hormonal replacement"],"prefix":"10.1093","volume":"184","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7790-6631","authenticated-orcid":true,"given":"Julia","family":"Rohayem","sequence":"first","affiliation":[{"name":"1Department of Clinical and Surgical Andrology, Center of Reproductive Medicine and Andrology, University of M\u00fcnster, M\u00fcnster, Nordrhein-Westfalen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lena Maria","family":"B\u00e4umer","sequence":"additional","affiliation":[{"name":"1Department of Clinical and Surgical Andrology, Center of Reproductive Medicine and Andrology, University of M\u00fcnster, M\u00fcnster, Nordrhein-Westfalen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Zitzmann","sequence":"additional","affiliation":[{"name":"1Department of Clinical and Surgical Andrology, Center of Reproductive Medicine and Andrology, University of M\u00fcnster, M\u00fcnster, Nordrhein-Westfalen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne","family":"Fricke-Otto","sequence":"additional","affiliation":[{"name":"2Department of Pediatric Endocrinology and Diabetes, Helios-Klinikum Krefeld, Krefeld, Nordrhein-Westfalen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaus","family":"Mohnike","sequence":"additional","affiliation":[{"name":"3Department of Pediatric Endocrinology and Diabetes, University of Magdeburg, Children's Hospital Magdeburg, Magdeburg, Sachsen-Anhalt, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bettina","family":"Gohlke","sequence":"additional","affiliation":[{"name":"4Department of Pediatric Endocrinology and Diabetes, Children's Hospital Bonn, University of Bonn, Bonn, Nordrhein-Westfalen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felix","family":"Reschke","sequence":"additional","affiliation":[{"name":"5Department of Pediatric Endocrinology and Diabetes, Children's Hospital Dresden, University of Dresden, Dresden, Sachsen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claus","family":"Jourdan","sequence":"additional","affiliation":[{"name":"6Department of Pediatric Endocrinology and Diabetes, Children's Hospital Herford, Herford, Nordrhein-Westfalen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Herman L","family":"M\u00fcller","sequence":"additional","affiliation":[{"name":"7Department of Pediatrics and Pediatric Hematology and Oncology, Children's Hospital Oldenburg, University of Oldenburg, Oldenburg, Niedersachsen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D\u00e9sir\u00e9e","family":"Dunstheimer","sequence":"additional","affiliation":[{"name":"8Department of Pediatric Endocrinology and Diabetes, Children's Hospital Augsburg, University of Augsburg, Augsburg, Bayern, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes","family":"Weigel","sequence":"additional","affiliation":[{"name":"9Pediatric Endocrinology practice, Augsburg, Bayern, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Norbert","family":"Jorch","sequence":"additional","affiliation":[{"name":"10Department of Pediatrics, Evangelisches Klinikum Bethel, Bielefeld, Nordrhein-Westfalen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elke","family":"M\u00fcller-Rossberg","sequence":"additional","affiliation":[{"name":"11Department of Pediatric Endocrinology and Diabetes, Children's Hospital, Esslingen, Baden-W\u00fcrttemberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erwin","family":"Lankes","sequence":"additional","affiliation":[{"name":"12Department of Pediatric Endocrinology and Diabetes, Children's Hospital, Charity, University of Berlin, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Imke","family":"G\u00e4tjen","sequence":"additional","affiliation":[{"name":"13Pediatric Endocrinology practice, Bremen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annette","family":"Richter-Unruh","sequence":"additional","affiliation":[{"name":"14Department of Pediatric Endocrinology and Diabetes, Children's Hospital, University of Bochum, Bochum, Nordrhein-Westfalen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Berthold P","family":"Hauffa","sequence":"additional","affiliation":[{"name":"15Department of Pediatric Endocrinology and Diabetes, Children's Hospital, University of Essen, Essen, Nordrhein-Westfalen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine","family":"Kliesch","sequence":"additional","affiliation":[{"name":"1Department of Clinical and Surgical Andrology, Center of Reproductive Medicine and Andrology, University of M\u00fcnster, M\u00fcnster, Nordrhein-Westfalen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aniko","family":"Krumbholz","sequence":"additional","affiliation":[{"name":"16Institute for Doping Analytics and Sports Biochemistry, Dresden, Sachsen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00fcrgen","family":"Br\u00e4mswig","sequence":"additional","affiliation":[{"name":"17Department of Pediatric Endocrinology and Diabetes, Children's Hospital, University of M\u00fcnster, M\u00fcnster, Nordrhein-Westfalen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","container-title":["European Journal of 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