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Rohit Barnabas Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Swati Jadhav Department of Endocrinology, Vydehi Institute of Medical Sciences and Research Centre, Bengaluru, India

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Anurag Ranjan Lila Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Sirisha Kusuma Boddu Consultant Pediatric Endocrinology & Diabetes, Rainbow Children’s Hospital, Hyderabad, India

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Saba Samad Memon Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Sneha Arya Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Samiksha Chandrashekhar Hegishte Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Manjiri Karlekar Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Virendra A Patil Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Vijaya Sarathi Department of Endocrinology, Vydehi Institute of Medical Sciences and Research Centre, Bengaluru, India

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Nalini S Shah Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Tushar Bandgar Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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age at presentation, patients with 46,XY were categorized as in minipubertal age (0 to ≤ 0.5 years), pre-pubertal age (> 0.5 to ≤ 9 years), pubertal age (> 9 years to ≤ 14 years) and post-pubertal age (> 14 years). A hCG-stimulated testosterone of <1

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Rachel K Rowe Phoenix Veterans Affairs Health Care System, Phoenix, Arizona, USA
BARROW Neurological Institute at Phoenix Children’s Hospital, Phoenix, Arizona, USA
Department of Child Health, University of Arizona College of Medicine – Phoenix, Phoenix, Arizona, USA

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Benjamin M Rumney BARROW Neurological Institute at Phoenix Children’s Hospital, Phoenix, Arizona, USA
Department of Child Health, University of Arizona College of Medicine – Phoenix, Phoenix, Arizona, USA
Department of Biology and Biochemistry, University of Bath, UK

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Hazel G May BARROW Neurological Institute at Phoenix Children’s Hospital, Phoenix, Arizona, USA
Department of Child Health, University of Arizona College of Medicine – Phoenix, Phoenix, Arizona, USA
Department of Biology and Biochemistry, University of Bath, UK

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Paska Permana Phoenix Veterans Affairs Health Care System, Phoenix, Arizona, USA

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P David Adelson BARROW Neurological Institute at Phoenix Children’s Hospital, Phoenix, Arizona, USA
Department of Child Health, University of Arizona College of Medicine – Phoenix, Phoenix, Arizona, USA
School of Biological and Health Systems Engineering, Arizona State University, Tempe, Arizona, USA

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S Mitchell Harman Phoenix Veterans Affairs Health Care System, Phoenix, Arizona, USA

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Jonathan Lifshitz Phoenix Veterans Affairs Health Care System, Phoenix, Arizona, USA
BARROW Neurological Institute at Phoenix Children’s Hospital, Phoenix, Arizona, USA
Department of Child Health, University of Arizona College of Medicine – Phoenix, Phoenix, Arizona, USA

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Theresa C Thomas Phoenix Veterans Affairs Health Care System, Phoenix, Arizona, USA
BARROW Neurological Institute at Phoenix Children’s Hospital, Phoenix, Arizona, USA
Department of Child Health, University of Arizona College of Medicine – Phoenix, Phoenix, Arizona, USA

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histological) were made at chronic time points postinjury to determine whether ongoing reparative processes could contribute to late-onset deficits. We hypothesized that diffuse TBI would lead to chronic deficiencies in CORT and testosterone, which are

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Yanling Cai Department of Nuclear Medicine, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, China

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Yan Yang Department of Nuclear Medicine, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, China

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Xiao Pang Department of Nuclear Medicine, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, China

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Suping Li Department of Nuclear Medicine, Affiliated Hospital of North Sichuan Medical College, Nanchong, Sichuan, China

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carcinoma, male, testis, sperm, follicle-stimulating hormone, thyroid neoplasm, gonadotropins, luteinizing hormone, testosterone, among others. The search terms were combined with the Boolean operators AND and OR. Inclusion and exclusion criteria for

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I Savchuk Department of Women’s and Children’s Health, Pediatric Endocrinology Unit, Karolinska Institute & University Hospital, Stockholm, Sweden

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M L Morvan LUNAM Université, École Nationale Vétérinaire, Agroalimentaire et de l’Alimentation, Nantes-Atlantique (Oniris), Laboratoire d’Étude des Résidus et Contaminants dans les Aliments (LABERCA), USC INRA 1329, Nantes, France

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J P Antignac LUNAM Université, École Nationale Vétérinaire, Agroalimentaire et de l’Alimentation, Nantes-Atlantique (Oniris), Laboratoire d’Étude des Résidus et Contaminants dans les Aliments (LABERCA), USC INRA 1329, Nantes, France

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K Gemzell-Danielsson Department of Obstetrics and Gynecology, Karolinska Institute & University Hospital, Stockholm, Sweden

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B Le Bizec LUNAM Université, École Nationale Vétérinaire, Agroalimentaire et de l’Alimentation, Nantes-Atlantique (Oniris), Laboratoire d’Étude des Résidus et Contaminants dans les Aliments (LABERCA), USC INRA 1329, Nantes, France

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O Söder Department of Women’s and Children’s Health, Pediatric Endocrinology Unit, Karolinska Institute & University Hospital, Stockholm, Sweden

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K Svechnikov Department of Women’s and Children’s Health, Pediatric Endocrinology Unit, Karolinska Institute & University Hospital, Stockholm, Sweden

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been reported that the HFA has the potential to produce testosterone from androstenedione by action of 17βHSD5 ( 5 ), similar to that observed in the adult adrenals, where this enzyme has been found to be expressed in zona reticularis ( 6 ). It is also

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Mojca Jensterle Department of Endocrinology, Diabetes and Metabolic Diseases, University Medical Centre, Ljubljana, Slovenia
University of Ljubljana, Faculty of Medicine, Ljubljana, Slovenia

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Nika Aleksandra Kravos Department of Endocrinology, Diabetes and Metabolic Diseases, University Medical Centre, Ljubljana, Slovenia
University of Ljubljana, Faculty of Medicine, Ljubljana, Slovenia

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Simona Ferjan Department of Endocrinology, Diabetes and Metabolic Diseases, University Medical Centre, Ljubljana, Slovenia
University of Ljubljana, Faculty of Medicine, Ljubljana, Slovenia

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Katja Goricar University of Ljubljana, Faculty of Medicine, Institute of Biochemistry, Pharmacogenetics Laboratory, Ljubljana, Slovenia

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Vita Dolzan University of Ljubljana, Faculty of Medicine, Institute of Biochemistry, Pharmacogenetics Laboratory, Ljubljana, Slovenia

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Andrej Janez Department of Endocrinology, Diabetes and Metabolic Diseases, University Medical Centre, Ljubljana, Slovenia
University of Ljubljana, Faculty of Medicine, Ljubljana, Slovenia

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pressure, menstrual frequency, fasting glucose, androstenedione, dehydroepiandrosterone sulphate (DHEAS), free and total testosterone, follicle-stimulating hormone (FSH), luteinizing hormone (LH) were identified at baseline and at every follow-up visit at 1

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Alexander Tacey Institute for Health and Sport (IHES), Victoria University, Melbourne, Victoria, Australia
Department of Medicine-Western Health, Australian Institute for Musculoskeletal Science (AIMSS), Melbourne Medical School, The University of Melbourne, Melbourne, Victoria, Australia

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Lewan Parker Institute for Health and Sport (IHES), Victoria University, Melbourne, Victoria, Australia
Institute for Physical Activity and Nutrition, Deakin University, Geelong, Victoria, Australia

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Bu B Yeap Medical School, University of Western Australia, and Department of Endocrinology and Diabetes, Fiona Stanley Hospital, Perth, Western Australia, Australia

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John Joseph PathWest Laboratory Medicine, Sir Charles Gairdner Hospital, Perth, Western Australia, Australia

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Ee M Lim PathWest Laboratory Medicine, Sir Charles Gairdner Hospital, Perth, Western Australia, Australia

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Andrew Garnham Institute for Health and Sport (IHES), Victoria University, Melbourne, Victoria, Australia

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David L Hare University of Melbourne and the Department of Cardiology, Austin Health, Melbourne, Victoria, Australia

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Tara Brennan-Speranza Department of Physiology and Bosch Institute for Medical Research, University of Sydney, Sydney, New South Wales, Australia

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Itamar Levinger Institute for Health and Sport (IHES), Victoria University, Melbourne, Victoria, Australia
Department of Medicine-Western Health, Australian Institute for Musculoskeletal Science (AIMSS), Melbourne Medical School, The University of Melbourne, Melbourne, Victoria, Australia

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reduction in circulating testosterone concentrations attributed to the inhibition of the hypothalamic–pituitary–testicular (HPT) axis ( 14 ). Exercise is not thought to interfere with the diurnal rhythm of testosterone production ( 15 ), and there may be a

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Rossella Cannarella Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy

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Andrea Crafa Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy

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Sandro La Vignera Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy

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Rosita A Condorelli Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy

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Aldo E Calogero Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy

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: puberty onset and progression, testicular volume, gonadotropin, and total testosterone serum levels, sperm parameters and fertility, micropenis. Methods Sources Data used to perform this systematic review were independently extracted by A C and

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Sebastian Franik Department of Obstetrics and Gynaecology, Radboudumc, Nijmegen, The Netherlands

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Kathrin Fleischer Department of Obstetrics and Gynaecology, Radboudumc, Nijmegen, The Netherlands

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Barbara Kortmann Department of Pediatric Urology, Radboudumc, Nijmegen, The Netherlands

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Nike M Stikkelbroeck Department of Internal Medicine, Radboudumc, Nijmegen, The Netherlands

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Kathleen D’Hauwers Department of Urology, Radboudumc, Nijmegen, The Netherlands

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Claire Bouvattier Department of Pediatric Endocrinology, Bicêtre Hospital, Paris Sud University, Paris, France

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Jolanta Slowikowska-Hilczer Department of Andrology and Reproductive Endocrinology, Medical University of Lodz, Lodz, Poland

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Solange Grunenwald Department of Endocrinology and Metabolic Disease, Centre Hospitalier Universitaire de Toulouse, Toulouse, France

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Tim van de Grift Departments of Plastic Surgery and Medical Psychology, Amsterdam UMC Location VUmc, Amsterdam, The Netherlands

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Audrey Cartault Department of Pediatrics, Hospital des Enfants, Toulouse, France

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Annette Richter-Unruh Kinderendokrinologie und Diabetologie, Universitätsklinikum Ruhr-Universität Bochum, Kinderklinik, Bochum, Germany

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Nicole Reisch Medizinische Klinik and Poliklinik IV, Department of Endocrinology, University Hospital Munich, Munich, Germany

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Ute Thyen Klinik fur Kinder- und Jugendmedizin, Universitat zu Lubeck, Lubeck, Germany

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Joanna IntHout Department for Health Evidence, Radboudumc, Nijmegen, The Netherlands

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Hedi L Claahsen-van der Grinten Department of Pediatric Endocrinology, Amalia Childrens Hospital, Radboudumc, Nijmegen, The Netherlands

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the dsd-LIFE Group
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the dsd-LIFE Group

azoospermia is found in about 90% of adult men with KS ( 2 ). Other frequent clinical symptoms include gynecomastia, a low sexual drive, small testes and hypogonadism due to impaired testosterone production ( 3 , 4 ). KS is also associated with

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Rafaella Sales de Freitas Departamento de Psicobiologia, Universidade Federal de São Paulo, São Paulo, Brazil

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Thiago F A França Departamento de Psicobiologia, Universidade Federal de São Paulo, São Paulo, Brazil

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Sabine Pompeia Departamento de Psicobiologia, Universidade Federal de São Paulo, São Paulo, Brazil

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). Apart from gonadal changes that result from this activation, which include the increased production of hormones such as testosterone and oestrogens, changes in genitalia in both sexes, breast growth in girls and facial hair growth, and voice changes in

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Srdjan Pandurevic Division of Endocrinology and Diabetes Prevention and Care, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy

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Ilaria Mancini Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy
Unit of Gynecology and Obstetrics, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Italy

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Dimitri Mitselman Division of Endocrinology and Diabetes Prevention and Care, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy

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Matteo Magagnoli Division of Endocrinology and Diabetes Prevention and Care, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy

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Rita Teglia Division of Endocrinology and Diabetes Prevention and Care, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy

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Roberta Fazzeri Division of Endocrinology and Diabetes Prevention and Care, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy

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Paola Dionese Division of Endocrinology and Diabetes Prevention and Care, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy

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Carolina Cecchetti Division of Endocrinology and Diabetes Prevention and Care, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy

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Massimiliamo Caprio Department of Human Sciences and Quality of Life Promotion, San Raffaele Roma Open University, Rome, Italy
Laboratory of Cardiovascular Endocrinology, IRCCS San Raffaele, Rome, Italy

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Costanzo Moretti Department of Systems Medicine, Unit of Endocrinology, University of Rome Tor Vergata, Rome, Italy

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Justyna Sicinska Dermatology Clinic of CSK MSWiA Hospital, Warsaw, Poland

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Alessandro Agostini Department of Experimental, Diagnostic, and Specialty Medicine, University of Bologna, Bologna, Italy

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Domenica Gazineo Teaching Hospital, S. Orsola Hospital, Bologna, Italy

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Lea Godino Teaching Hospital, S. Orsola Hospital, Bologna, Italy

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Ignacio Sajoux Epigenomics in Endocrinology and Nutrition Group, Instituto de Investigacion Sanitaria (IDIS), Complejo Hospitalario Universitario de Santiago (CHUS/SERGAS), Spain
Medical Department Pronokal Group, Barcelona, Spain

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Flaminia Fanelli Division of Endocrinology and Diabetes Prevention and Care, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy

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Cristina M Meriggiola Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy
Unit of Gynecology and Obstetrics, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Italy

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Uberto Pagotto Division of Endocrinology and Diabetes Prevention and Care, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy

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Alessandra Gambineri Division of Endocrinology and Diabetes Prevention and Care, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
Department of Medical and Surgical Sciences (DIMEC), Alma Mater Studiorum University of Bologna, Bologna, Italy

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-binding globulin (SHBG) were immediately processed at a laboratory in Bologna (Laboratorio Unico Metropolitano). Additionally, serum was obtained by a 5 mL blood sample and stored at −20°C for later measurement of androgens (testosterone, androstenedione, DHEA, 17

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