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Soraya Puglisi Internal Medicine 1, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy

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Paola Perotti Internal Medicine 1, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy

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Mattia Barbot Endocrinology Unit, Department of Medicine DIMED, University of Padua, Padua, Italy

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Paolo Cosio Internal Medicine 1, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy

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Carla Scaroni Endocrinology Unit, Department of Medicine DIMED, University of Padua, Padua, Italy

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Antonio Stigliano Endocrinology Unit, Department of Clinical and Molecular Medicine, Sant’Andrea Hospital University of Rome, Rome, Italy

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Pina Lardo Endocrinology Unit, Department of Clinical and Molecular Medicine, Sant’Andrea Hospital University of Rome, Rome, Italy

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Valentina Morelli Endocrinology Unit, Department of Clinical Sciences and Community Health, University of Milan and Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milano, Italy

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Elisa Polledri Department of Clinical Sciences and Community Health, Laboratory of Toxicology, University of Milan and Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milan, Italy

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Iacopo Chiodini Endocrinology Unit, Department of Clinical Sciences and Community Health, University of Milan and Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Milano, Italy

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Giuseppe Reimondo Internal Medicine 1, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy

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Anna Pia Internal Medicine 1, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy

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Massimo Terzolo Internal Medicine 1, Department of Clinical and Biological Sciences, University of Turin, Turin, Italy

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). In particular, metyrapone, which affects cortisol secretion by inhibiting the 11β-hydroxylase enzyme (CYP11B1) ( 5 , 6 ), has a solid reputation of efficacy and entered clinical use since his introduction in 1958 ( 7 ); however, the drug has never

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I Azzam Institute of Endocrinology, Metabolism and Hypertension, Tel Aviv-Sourasky Medical Center, Tel Aviv, Israel

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S Gilad Institute of Endocrinology, Metabolism and Hypertension, Tel Aviv-Sourasky Medical Center, Tel Aviv, Israel

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R Limor Institute of Endocrinology, Metabolism and Hypertension, Tel Aviv-Sourasky Medical Center, Tel Aviv, Israel

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N Stern Institute of Endocrinology, Metabolism and Hypertension, Tel Aviv-Sourasky Medical Center, Tel Aviv, Israel
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

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Y Greenman Institute of Endocrinology, Metabolism and Hypertension, Tel Aviv-Sourasky Medical Center, Tel Aviv, Israel
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

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subjects pre-treated with metyrapone ( 28 ). In contrast to the expanding knowledge on ghrelin’s biological functions, the regulation of ghrelin’s synthesis and secretion is less well characterized, although involvement of the autonomic nervous system

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M S Elston Department of Endocrinology, Waikato Hospital, Hamilton, New Zealand
Waikato Clinical Campus, University of Auckland, Hamilton, New Zealand

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V B Crawford Department of Endocrinology, Waikato Hospital, Hamilton, New Zealand

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M Swarbrick Department of Radiology, Waikato Hospital, Hamilton, New Zealand

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M S Dray Department of Pathology, Waikato Hospital, Hamilton, New Zealand

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M Head Department of Oncology, Tauranga Hospital, Tauranga, New Zealand

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J V Conaglen Waikato Clinical Campus, University of Auckland, Hamilton, New Zealand

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of the diagnosis of prostate neuroendocrine carcinoma, urgent treatment with inhibitors of adrenal steroidogenesis was initiated (ketoconazole, 1.2 g daily in divided doses and metyrapone 6 g daily in divided doses), along with mineralocorticoid

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Agata Hanna Bryk-Wiązania Chair and Department of Endocrinology, Jagiellonian University Medical College, Krakow, Poland
Department of Endocrinology, Oncological Endocrinology and Nuclear Medicine, University Hospital, Krakow, Poland

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Mari Minasyan Department of Endocrinology, Oncological Endocrinology and Nuclear Medicine, University Hospital, Krakow, Poland

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Alicja Hubalewska-Dydejczyk Chair and Department of Endocrinology, Jagiellonian University Medical College, Krakow, Poland
Department of Endocrinology, Oncological Endocrinology and Nuclear Medicine, University Hospital, Krakow, Poland

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Aleksandra Gilis-Januszewska Chair and Department of Endocrinology, Jagiellonian University Medical College, Krakow, Poland
Department of Endocrinology, Oncological Endocrinology and Nuclear Medicine, University Hospital, Krakow, Poland

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)  Vertebral osteoporosis 2 (22)  Unusual infections 1 (11) *ketoconazole or metyrapone, or osilodrostat or etomidate; **denotes ketoconazole, or metyrapone, or mitotane (in the case of adrenal carcinoma). TSS, transsphenoidal

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Emmanuelle Motte UFR des Sciences de la Santé, Simone Veil, Université Versailles St-Quentin en Yvelines, Montigny le Bretonneux, France
Assistance Publique Hôpitaux de Paris (APHP), Department of Endocrinology and Diabetes for Children, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France

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Anya Rothenbuhler Assistance Publique Hôpitaux de Paris (APHP), Department of Endocrinology and Diabetes for Children, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France
APHP, Plateforme d’Expertise Maladies Rares Paris Sud, Bicêtre Paris Sud Hospital, Le Kremlin Bicêtre, France

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Stephan Gaillard Department of Neurosurgery, Foch Hospital, Suresnes, France

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Najiba Lahlou APHP, Department of Hormonal Biology, Cochin Hospital, Paris, France

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Cécile Teinturier Assistance Publique Hôpitaux de Paris (APHP), Department of Endocrinology and Diabetes for Children, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France
APHP, Plateforme d’Expertise Maladies Rares Paris Sud, Bicêtre Paris Sud Hospital, Le Kremlin Bicêtre, France

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Régis Coutant Department of Pediatric Endocrinology, Angers University Hospital, Angers, France

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Agnès Linglart Assistance Publique Hôpitaux de Paris (APHP), Department of Endocrinology and Diabetes for Children, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France
APHP, Plateforme d’Expertise Maladies Rares Paris Sud, Bicêtre Paris Sud Hospital, Le Kremlin Bicêtre, France

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and metyrapone. Mitotane is approved for the treatment of adrenocortical carcinoma. It has been used as a cortisol-lowering drug for the treatment of CD in adults ( 27 , 28 , 29 , 30 , 31 ). A case report describes the use of mitotane for 18

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Gavin P Vinson School of Biological and Chemical Sciences, Queen Mary University of London, London E1 4NS, UK

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Caroline H Brennan School of Biological and Chemical Sciences, Queen Mary University of London, London E1 4NS, UK

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Impaired HPA feedback in cocaine habituated animals 28, 29 Synthesis blocked By metyrapone 50 mg/kg Reduced psychomotor effects of cocaine, and reduced reinstatement 196 By metyrapone 100 mg/kg Reduced psychomotor effects of cocaine, and reduced

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Ferdinand Roelfsema Department of Internal Medicine, Leiden University Medical Center, Leiden, The Netherlands

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Diana van Heemst Department of Internal Medicine, Section Gerontology and Geriatrics, Leiden University Medical Center, Leiden, The Netherlands

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Ali Iranmanesh Endocrine Section, Medical Service, Salem Veterans Affairs Medical Center, Salem, Virginia, USA

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Paul Takahashi Primary Care Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA

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Rebecca Yang Endocrine Research Unit, Mayo Medical and Graduate Schools, Clinical Translational Research Center, Mayo Clinic, Rochester, Minnesota, USA

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Johannes D Veldhuis Endocrine Research Unit, Mayo Medical and Graduate Schools, Clinical Translational Research Center, Mayo Clinic, Rochester, Minnesota, USA

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their partners ( 8 ). In another study, hydrocortisone administration during the quiescent phase in metyrapone-clamped volunteers reduced insulin sensitivity and glucose tolerance ( 31 ). However, in this study, serum cortisol levels reached about 800

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Pablo Abellán-Galiana Department of Endocrinology, Hospital General Universitari de Castelló, Castellón, Spain
Department of Medicine, Universidad Cardenal Herrera-CEU, CEU Universities, Castellón, Spain

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Carmen Fajardo-Montañana Department of Endocrinology, Hospital Universitario de la Ribera, Alzira, Spain

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Pedro Riesgo-Suárez Department of Neurosurgery, Hospital Universitario de la Ribera, Alzira, Spain

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Marcelino Pérez-Bermejo Department of Nursing, Universidad Católica de Valencia, Valencia, Spain

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Celia Ríos-Pérez Centro de Salud Tavernes de la Valldigna, Hospital Comarcal Francesc de Borja, Gandía, Spain

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José Gómez-Vela Department of Endocrinology, Hospital Universitario de la Ribera, Alzira, Spain

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results of postoperative basal cortisol, among which are: corticotropin-releasing hormone test ( 27 ), metyrapone test ( 28 ), desmopressin test ( 29 , 30 ) and dexamethasone suppression test after desmopressin ( 31 ). For the interpretation of these last

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Elena Valassi Endocrinology Department, Germans Trias i Pujol Hospital and Research Institute, Badalona, Barcelona, Spain
IRCCS, Istituto Auxologico Italiano, Milan, Italy
Department of Medical Biotechnology and Translational Medicine, University of Milan, Milan, Italy

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Iacopo Chiodini IRCCS, Istituto Auxologico Italiano, Milan, Italy
Department of Medical Biotechnology and Translational Medicine, University of Milan, Milan, Italy

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Richard A Feelders Division of Endocrinology, Erasmus Medical Centre, Rotterdam, Netherlands

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Cornelie D Andela Division of Endocrinology and Centre for Endocrine Tumours, Leiden University Medical Centre, Leiden, Netherlands

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Margueritta Abou-Hanna HRA Pharma Rare Diseases, Châtillon, France

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Sarah Idres HRA Pharma Rare Diseases, Châtillon, France

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Antoine Tabarin Department of Endocrinology and INSERM U862 University and CHU of Bordeaux, Pessac, France

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Ceccato F Zilio M Barbot M Albiger N Antonelli G Plebani M Watutantrige-Fernando S Sabbadin C Boscaro M Scaroni C . Metyrapone treatment in Cushing’s syndrome: a real-life study . Endocrine 2018 62 701 – 711 . ( https://doi.org/10

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M Cherenko Department of Medicine, Division of Endocrinology, Leiden University Medical Centre, Leiden, Netherlands

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N M Appelman-Dijkstra Department of Medicine, Division of Endocrinology, Leiden University Medical Centre, Leiden, Netherlands

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A L Priego Zurita Department of Medicine, Division of Endocrinology, Leiden University Medical Centre, Leiden, Netherlands

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N R Biermasz Department of Medicine, Division of Endocrinology, Leiden University Medical Centre, Leiden, Netherlands

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O M Dekkers Department of Medicine, Division of Endocrinology, Leiden University Medical Centre, Leiden, Netherlands

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F A Klok Department of Medicine, Division of Thrombosis and Haemostasis, Leiden University Medical Centre, Leiden, Netherlands

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N Reisch Department of Medicine IV, LMU University Hospital, LMU Munich, Munich, Germany

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A Aulinas Department of Endocrinology, Fundacio de Gestio Sanitaria Hospital de la Santa Creu i Sant Pau, IR-SantPau and CIBERER Unit 747 (ISCIII), Barcelona, Spain

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B Biagetti Department of Endocrinology, Hospital Universitari Vall d’Hebron, Barcelona, Spain

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S Cannavo Endocrine Unit, University Hospital AOU Policlinico G. Martino, Messina, Italy

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L Canu University Hospital Florence Careggi, Florence, Italy

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M Detomas Department of Internal Medicine, University Hospital Würzburg, Wuerzburg, Germany

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F Devuyst Department of Endocrinology, Hôpital Universitaire de Bruxelles, Hôpital Erasme, Brussels, Belgium

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H Falhammar Department of Endocrinology, Karolinska University Hospital and Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden

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R A Feelders Department of Internal Medicine, Division of Endocrinology, Erasmus MC, Rotterdam, Netherlands

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F Ferrau Endocrine Unit, University Hospital AOU Policlinico G. Martino, Messina, Italy

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F Gatto IRCCS Ospedale Policlinico San Martino, Genova, Genoa, Italy

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C Grasselli Cardiovascular Medicine Unit, AUSL-IRCCS, Reggio Emilia, Italy

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P van Houten Department of Internal Medicine, Division of Endocrinology, Radboud University Medical Center, Nijmegen, Netherlands

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C Hoybye Department of Endocrinology, Karolinska University Hospital and Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden

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A M Isidori Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy

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A Kyrilli Department of Endocrinology, Hôpital Universitaire de Bruxelles, Hôpital Erasme, Brussels, Belgium

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P Loli Division of Endocrinology, San Raffaele Vita-Salute University, IRCCS San Raffaele Hospital Milan, Italy

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D Maiter Department of Endocrinology, Cliniques universitaires Saint-Luc – UCLouvain, Brussels, Belgium

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E Nowak Department of Medicine IV, LMU University Hospital, LMU Munich, Munich, Germany

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R Pivonello Dipartimento di Medicina Clinica e Chirurgia, Sezione di Endocrinologia, Diabetologia, Andrologia e Nutrizione, Università “Federico II” di Napoli, Naples, Italy

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O Ragnarsson Sahlgrenska Academy, Wallenberg Centre for Molecular and Translational Medicine, Institute of Medicine (O.R.), University of Gothenburg, Sweden

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R V Steenaard Department of Internal Medicine, Máxima MC, Veldhoven, Netherlands

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N Unger University Hospital Essen, Department of Endocrinology, Diabetes and Metabolism, Essen, Germany

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A van de Ven Department of Internal Medicine, Division of Endocrinology, Radboud University Medical Center, Nijmegen, Netherlands

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S M Webb Department of Endocrinology, Fundacio de Gestio Sanitaria Hospital de la Santa Creu i Sant Pau, IR-SantPau and CIBERER Unit 747 (ISCIII), Barcelona, Spain

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D Yeste Pediatric Endocrinology Service, Hospital Universitari Vall d’Hebron, Universitat Autònoma de Barcelona, Barcelona, Spain. CIBER Enfermedades Raras, Instituto Carlos III, Madrid, Spain

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S F Ahmed Department of Medicine, Division of Endocrinology, Leiden University Medical Centre, Leiden, Netherlands
University of Glasgow, Office for Rare Conditions, Glasgow, UK
University of Glasgow, Developmental Endocrinology Research Group, Royal Hospital for Children, Glasgow, UK

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A M Pereira Department of Endocrinology & Metabolism, Amsterdam University Medical Centre, Amsterdam, Noord-Holland, Netherlands

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to CD. Cortisol-lowering medical treatment Eighty-four patients (38%) received pre-surgical cortisol-lowering medical treatment, the majority receiving metyrapone (68%) or ketoconazole (30%). Other used agents were osilodrostat (8%), mitotane

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