Hormonal control during infancy and testicular adrenal rest tumor development in males with congenital adrenal hyperplasia: a retrospective multicenter cohort study.

Autor:	Schröder MAM; Department of Pediatrics, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.; Department of Laboratory Medicine, Radboudumc Graduate School, Radboud University Medical Center, Nijmegen, The Netherlands., Neacşu M; Department of Laboratory Medicine, Radboudumc Graduate School, Radboud University Medical Center, Nijmegen, The Netherlands., Adriaansen BPH; Department of Pediatrics, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.; Department of Laboratory Medicine, Radboudumc Graduate School, Radboud University Medical Center, Nijmegen, The Netherlands., Sweep FCGJ; Department of Laboratory Medicine, Radboudumc Graduate School, Radboud University Medical Center, Nijmegen, The Netherlands., Ahmed SF; Developmental Endocrinology Research Group, University of Glasgow, Glasgow, United Kingdom.; Office of Rare Conditions, University of Glasgow, Glasgow, United Kingdom., Ali SR; Developmental Endocrinology Research Group, University of Glasgow, Glasgow, United Kingdom.; Office of Rare Conditions, University of Glasgow, Glasgow, United Kingdom., Bachega TASS; Laboratory of Hormones and Molecular Genetics-LIM 42, Department of Endocrinology and Metabolism, University of Sao Paulo, Sao Paulo, Brazil., Baronio F; Department Hospital of Woman and Child, Pediatric Unit, IRCCS AOU di Bologna, Policlinico di S.Orsola, Bologna, Italy., Birkebæk NH; Department of Pediatrics and Steno Diabetes Center Aarhus, Aarhus University Hospital, Aarhus, Denmark., de Bruin C; Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands., Bonfig W; Department of Pediatrics, Technical University Munich, Munich, Germany.; Department of Pediatrics, Klinikum Wels-Grieskirchen, Wels, Austria., Bryce J; Office of Rare Conditions, University of Glasgow, Glasgow, United Kingdom., Clemente M; Pediatric Endocrinology Unit, Hospital Vall d'Hebron, Autonomous University of Barcelona, CIBERER, Barcelona, Spain., Cools M; Pediatric Endocrinology, Internal Medicine and Pediatric Research Unit, University Hospital Ghent, Ghent University, Ghent, Belgium., Elsedfy H; Pediatrics Department, Ain Shams University, Cairo, Egypt., Globa E; Ukrainian Research Center of Endocrine Surgery, Endocrine Organs and Tissue Transplantation, MOH of Ukraine, Kyiv, Ukraine., Guran T; Pediatric Endocrinology and Diabetes, Marmara University, Istanbul, Turkey., Güven A; Baskent University Medical Faculty, Istanbul Hospital, Pediatrics Department, Ain Shams University, Cairo, Egypt., Amr NH; Pediatrics Department, Ain Shams University, Cairo, Egypt., Janus D; Department of Pediatric and Adolescent Endocrinology, Institute of Pediatrics, Jagiellonian University Medical College, and Children's University Hospital, Krakow, Poland., Taube NL; Department of Pediatrics, Division of Endocrinology, University Children's Hospital Zurich, University of Zurich, Zurich, Switzerland., Markosyan R; Yerevan State Medical University, Yerevan, Armenia., Miranda M; Laboratory of Hormones and Molecular Genetics-LIM 42, Department of Endocrinology and Metabolism, University of Sao Paulo, Sao Paulo, Brazil., Poyrazoğlu Ş; İstanbul Faculty of Medicine, Unit of Pediatric Endocrinology, İstanbul University, İstanbul, Turkey., Rees A; Neuroscience and Mental Health Research Institute, Cardiff University, Cardiff, United Kingdom., Salerno M; Pediatric Endocrine Unit, Department of Translational Medical Sciences, University of Naples Federico II, Naples, Italy., Stancampiano MR; Department of Pediatrics, Endocrine Unit, IRCCS San Raffaele Scientific Institute, Endo-ERN Center for Rare Endocrine Conditions, Milan, Italy., Vieites A; Centro de Investigaciones Endocrinológicas Buenos Aires, Buenos Aires, Argentina., de Vries L; The Jesse Z and Sara Lea Shafer Institute for Endocrinology and Diabetes, National Center for Childhood Diabetes, Schneider Children's Medical Center of Israel and Felsenstein Medical Research Center at Petach Tikva, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel., Yavas Abali Z; Pediatric Endocrinology and Diabetes, Marmara University, Istanbul, Turkey., Span PN; Radiotherapy & OncoImmunology Laboratory, Department of Radiation Oncology, Radboudumc Graduate School, Radboud University Medical Center, Nijmegen, The Netherlands., Claahsen-van der Grinten HL; Department of Pediatrics, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
Jazyk:	angličtina
Zdroj:	European journal of endocrinology [Eur J Endocrinol] 2023 Oct 17; Vol. 189 (4), pp. 460-468.
DOI:	10.1093/ejendo/lvad143
Abstrakt:	Importance: Testicular adrenal rest tumors (TARTs), often found in male patients with congenital adrenal hyperplasia (CAH), are benign lesions causing testicular damage and infertility. We hypothesize that chronically elevated adrenocorticotropic hormone exposure during early life may promote TART development. Objective: This study aimed to examine the association between commencing adequate glucocorticoid treatment early after birth and TART development. Design and Participants: This retrospective multicenter (n = 22) open cohort study collected longitudinal clinical and biochemical data of the first 4 years of life using the I-CAH registry and included 188 male patients (median age 13 years; interquartile range: 10-17) with 21-hydroxylase deficiency (n = 181) or 11-hydroxylase deficiency (n = 7). All patients underwent at least 1 testicular ultrasound. Results: TART was detected in 72 (38%) of the patients. Prevalence varied between centers. When adjusted for CAH phenotype, a delayed CAH diagnosis of >1 year, compared with a diagnosis within 1 month of life, was associated with a 2.6 times higher risk of TART diagnosis. TART onset was not predicted by biochemical disease control or bone age advancement in the first 4 years of life, but increased height standard deviation scores at the end of the 4-year study period were associated with a 27% higher risk of TART diagnosis. Conclusions and Relevance: A delayed CAH diagnosis of >1 year vs CAH diagnosis within 1 month after birth was associated with a higher risk of TART development, which may be attributed to poor disease control in early life. Competing Interests: Conflict of interest: H.L.C.-v.d.G. is on the editorial board of EJE. She was not involved in the review or editorial process for this paper, on which she is listed as an author. H.L.C.-v.d.G. is also involved in clinical trials in CAH sponsored by Spruce Biosciences. S.F.A. has received an unrestricted education grant from Neurocrine Biosciences and acted as a consultant to Novo Nordisk. A.R. is involved in clinical trials in CAH sponsored by Diurnal Ltd and Neurocrine Biosciences. All other authors declared to have no conflict of interest. (© The Author(s) 2023. Published by Oxford University Press on behalf of European Society of Endocrinology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)
Databáze:	MEDLINE
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