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Natacha Driessens
Natacha Driessens
Université libre de Bruxelles (ULB), Hôpital Universitaire de Bruxelles (HUB), CUB Hôpital Erasme, Department of Endocrinology, Route de Lennik, Brussels, Belgium
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Madhu Prasai
Madhu Prasai
Université libre de Bruxelles (ULB), Hôpital Universitaire de Bruxelles (HUB), CUB Hôpital Erasme, Department of Endocrinology, Route de Lennik, Brussels, Belgium
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Orsalia Alexopoulou
Orsalia Alexopoulou
Department of Endocrinology, Cliniques Universitaires Saint Luc, Brussels, Belgium
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Christophe De Block
Christophe De Block
Department of Endocrinology-Diabetology-Metabolism, Universitair Ziekenhuis Antwerpen & University of Antwerp, Drie Eikenstraat, Edegem, Belgium
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Eva Van Caenegem
Eva Van Caenegem
Department of Endocrinology, Academisch Ziekenhuis Sint-Jan Brugge – Oostende AV, Ruddershove, Brugge, Belgium
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Guy T’Sjoen
Guy T’Sjoen
Department of Endocrinology, Ghent Universitary Hospital, C. Heymanslaan, Gent, Belgium
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Frank Nobels
Frank Nobels
Department of Endocrinology, Onze-Lieve Vrouw Ziekenhuis, Moorselbaan, Aalst, Belgium
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Christophe Ghys
Christophe Ghys
Department of Endocrinology, Universitair Ziekenhuis Brussel, Laarbeeklaan, Brussels, Belgium
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Laurent Vroonen
Laurent Vroonen
Department of Endocrinology, Cliniques Universitaires de Liège, Avenue de l’hôpital, Liège, Belgium
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Corinne Jonas
Corinne Jonas
Department of Endocrinology, CHU UCL Namur - Godinne, Avenue Docteur Gaston Thérasse, Yvoir, Belgium
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Bernard Corvilain
Bernard Corvilain
Université libre de Bruxelles (ULB), Hôpital Universitaire de Bruxelles (HUB), CUB Hôpital Erasme, Department of Endocrinology, Route de Lennik, Brussels, Belgium
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Dominique Maiter
Dominique Maiter
Department of Endocrinology, Cliniques Universitaires Saint Luc, Brussels, Belgium
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Introduction Primary adrenal insufficiency (PAI) is a rare disease, most commonly of auto-immune origin. There is a global variation in prevalence from 80 to 145 per million in Europe, with the highest case numbers reported in Scandinavian
Kathrin Zopf
Kathrin Zopf
Department of Endocrinology, Diabetes and Nutrition, Charité – Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany
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Kathrin R Frey
Kathrin R Frey
Department of Medicine I, Endocrine and Diabetes Unit, University Hospital, University of Würzburg, Würzburg, Germany
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Tina Kienitz
Tina Kienitz
Department of Endocrinology, Diabetes and Nutrition, Charité – Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany
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Manfred Ventz
Manfred Ventz
Department of Endocrinology, Diabetes and Nutrition, Charité – Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany
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Britta Bauer
Britta Bauer
Endocrinology in Charlottenburg, Berlin, Germany
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Marcus Quinkler
Marcus Quinkler
Endocrinology in Charlottenburg, Berlin, Germany
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AC might be influenced by the Bcl I polymorphism. Therefore, we included 72 patients with adrenal insufficiency (47 patients with primary adrenal insufficiency (PAI) and 25 patients with congenital adrenal hyperplasia (CAH)) in a prospective
Lia Ferreira
Lia Ferreira
Department of Endocrinology, Centro Hospitalar do Porto, Porto, Portugal
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João Silva
João Silva
Department of Endocrinology, Hospital das Forças Armadas, Lisboa, Portugal
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Susana Garrido
Susana Garrido
Department of Endocrinology, Centro Hospitalar Tâmega e Sousa, Porto, Portugal
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Carlos Bello
Carlos Bello
Department of Endocrinology, Centro Hospitalar Lisboa Ocidental, Lisboa, Portugal
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Diana Oliveira
Diana Oliveira
Department of Endocrinology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
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Hélder Simões
Hélder Simões
Department of Endocrinology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisboa, Portugal
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Isabel Paiva
Isabel Paiva
Department of Endocrinology, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
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Joana Guimarães
Joana Guimarães
Department of Endocrinology, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal
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Marta Ferreira
Marta Ferreira
Department of Endocrinology, Centro Hospitalar de Leiria, Leiria, Portugal
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Teresa Pereira
Teresa Pereira
Department of Endocrinology, Centro Hospitalar de Leiria, Leiria, Portugal
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Rita Bettencourt-Silva
Rita Bettencourt-Silva
Department of Endocrinology, Centro Hospitalar de São João, Porto, Portugal
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Ana Filipa Martins
Ana Filipa Martins
Department of Endocrinology, Centro Hospitalar Lisboa Norte, Lisboa, Portugal
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Tiago Silva
Tiago Silva
Department of Endocrinology, Hospital Garcia da Orta, Lisboa, Portugal
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Vera Fernandes
Vera Fernandes
Department of Endocrinology, Hospital de Braga, Braga, Portugal
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Maria Lopes Pereira
Maria Lopes Pereira
Department of Endocrinology, Hospital de Braga, Braga, Portugal
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Adrenal Tumors Study Group of the Portuguese Society of Endocrinology
Adrenal Tumors Study Group of the Portuguese Society of Endocrinology
Department of Endocrinology, Centro Hospitalar do Porto, Porto, Portugal
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Introduction Addison disease (AD) or primary adrenal insufficiency (PAI) is a life-threatening disease that results from bilateral destruction or dysfunction of the adrenal cortex ( 1 ). PAI is a rare disease with a reported prevalence of
Gregory Knowles
Gregory Knowles
Walsall Manor Hospital, Walsall, UK
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Emily Warmington
Emily Warmington
College of Medical and Dental Sciences, University of Birmingham, Birmingham, UK
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Lisa M Shepherd
Lisa M Shepherd
Department of Endocrinology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
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Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
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Jonathan M Hazlehurst
Jonathan M Hazlehurst
Department of Endocrinology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
Institute of Applied Health Research, University of Birmingham, Birmingham, UK
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Institute of Applied Health Research, University of Birmingham, Birmingham, UK
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Anne de Bray
Anne de Bray
Department of Endocrinology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
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Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
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Helena Gleeson
Helena Gleeson
Department of Endocrinology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
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Wiebke Arlt
Wiebke Arlt
Department of Endocrinology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
Medical Research Council London Institute of Medical Sciences, London, UK
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Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
Medical Research Council London Institute of Medical Sciences, London, UK
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Alessandro Prete
Alessandro Prete
Department of Endocrinology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
NIHR Birmingham Biomedical Research Centre, University of Birmingham and University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
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Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK
NIHR Birmingham Biomedical Research Centre, University of Birmingham and University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
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Introduction Primary adrenal insufficiency (PAI) is characterised by inadequate steroid hormone secretion caused by intrinsic adrenal cortex disease ( 1 ). The most common causes of PAI are Addison’s disease (AD), usually resulting from
Kathrin R Frey
Kathrin R Frey
Department of Medicine I, Endocrine and Diabetes Unit, University Hospital, University of Würzburg, Würzburg, Germany
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Tina Kienitz
Tina Kienitz
Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany
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Julia Schulz
Julia Schulz
Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany
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Manfred Ventz
Manfred Ventz
Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany
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Kathrin Zopf
Kathrin Zopf
Charité – Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany
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Marcus Quinkler
Marcus Quinkler
Endocrinology in Charlottenburg, Berlin, Germany
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Introduction In states of adrenal insufficiency (AI), such as primary adrenal insufficiency (PAI) and congenital adrenal hyperplasia (CAH), glucocorticoids (GCs) are given in low doses as hormone replacement therapy. However, the daily intake
Deirdre Green
Deirdre Green
Academic Department of Endocrinology, Beaumont Hospital and the Royal College of Surgeons in Ireland, Dublin
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Rosemary Dineen
Rosemary Dineen
Academic Department of Endocrinology, Beaumont Hospital and the Royal College of Surgeons in Ireland, Dublin
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Michael W O’Reilly
Michael W O’Reilly
Academic Department of Endocrinology, Beaumont Hospital and the Royal College of Surgeons in Ireland, Dublin
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Mark Sherlock
Mark Sherlock
Academic Department of Endocrinology, Beaumont Hospital and the Royal College of Surgeons in Ireland, Dublin
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Introduction Adrenal insufficiency (AI) is characterised by inadequate adrenal corticosteroid hormone production and is classified as primary, secondary or tertiary ( 1 ). Primary adrenal insufficiency (PAI) is caused by pathological processes
Avinaash Maharaj
Avinaash Maharaj
Centre for Endocrinology, John Vane Science Centre, Queen Mary University of London, London, UK
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Ruth Kwong
Ruth Kwong
Centre for Endocrinology, John Vane Science Centre, Queen Mary University of London, London, UK
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Jack Williams
Jack Williams
Centre for Endocrinology, John Vane Science Centre, Queen Mary University of London, London, UK
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Christopher Smith
Christopher Smith
Centre for Endocrinology, John Vane Science Centre, Queen Mary University of London, London, UK
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Helen Storr
Helen Storr
Centre for Endocrinology, John Vane Science Centre, Queen Mary University of London, London, UK
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Ruth Krone
Ruth Krone
Birmingham Children’s Hospital, Birmingham, UK
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Debora Braslavsky
Debora Braslavsky
Centro de Investigaciones Endocrinológicas ‘Dr. Cesar Bergadá’ (CEDIE) – CONICET – FEI – División de Endocrinología, Hospital de Niños ‘Ricardo Gutiérrez’, Buenos Aires, Argentina
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Maria Clemente
Maria Clemente
Paediatric Endocrinology, Growth and Development Research Unit, Vall d’Hebron Research Institute (VHIR), Hospital Vall d’Hebron, CIBERER, Instituto de Salud Carlos III, Barcelona, Spain
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Nanik Ram
Nanik Ram
Department of Endocrinology, The Aga Khan University Hospital, Karachi, Pakistan
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Indraneel Banerjee
Indraneel Banerjee
Department of Paediatric Endocrinology, Royal Manchester Children’s Hospital, Manchester, UK
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Semra Çetinkaya
Semra Çetinkaya
Health Sciences University, Dr. Sami Ulus Obstetrics and Gynaecology, Children’s Health and Disease Education and Research Hospital, Ankara, Turkey
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Federica Buonocore
Federica Buonocore
Genetics and Genomic Medicine Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK
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Tülay Güran
Tülay Güran
Department of Paediatric Endocrinology and Diabetes, Marmara University, School of Medicine, Istanbul, Turkey
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John C Achermann
John C Achermann
Genetics and Genomic Medicine Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, University College London, London, UK
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Louise Metherell
Louise Metherell
Centre for Endocrinology, John Vane Science Centre, Queen Mary University of London, London, UK
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Rathi Prasad
Rathi Prasad
Centre for Endocrinology, John Vane Science Centre, Queen Mary University of London, London, UK
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which primary adrenal insufficiency (PAI) and steroid-resistant nephrotic syndrome (SRNS) necessitating renal transplantation predominate ( 1 , 2 , 3 , 4 ). Additional clinical features including ichthyosis, progressive neurological disease, primary
Nilesh Lomte
Nilesh Lomte
Department of Endocrinology, Seth G S Medical College, KEM Hospital, Mumbai, Maharashtra, India
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Tushar Bandgar
Tushar Bandgar
Department of Endocrinology, Seth G S Medical College, KEM Hospital, Mumbai, Maharashtra, India
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Shruti Khare
Shruti Khare
Department of Endocrinology, Seth G S Medical College, KEM Hospital, Mumbai, Maharashtra, India
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Swati Jadhav
Swati Jadhav
Department of Endocrinology, Seth G S Medical College, KEM Hospital, Mumbai, Maharashtra, India
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Anurag Lila
Anurag Lila
Department of Endocrinology, Seth G S Medical College, KEM Hospital, Mumbai, Maharashtra, India
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Manjunath Goroshi
Manjunath Goroshi
Department of Endocrinology, Seth G S Medical College, KEM Hospital, Mumbai, Maharashtra, India
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Rajeev Kasaliwal
Rajeev Kasaliwal
Department of Endocrinology, Seth G S Medical College, KEM Hospital, Mumbai, Maharashtra, India
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Kranti Khadilkar
Kranti Khadilkar
Department of Endocrinology, Seth G S Medical College, KEM Hospital, Mumbai, Maharashtra, India
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Nalini S Shah
Nalini S Shah
Department of Endocrinology, Seth G S Medical College, KEM Hospital, Mumbai, Maharashtra, India
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primary adrenal insufficiency: an Endocrine Society clinical practice guideline . Journal of Clinical Endocrinology and Metabolism 2016 101 364 – 389 . ( doi:10.1210/jc.2015-1710 ) 7 El-Hefnawy AS El Garba M Osman Y Eraky I El
Hanna F Nowotny
Hanna F Nowotny
Medizinische Klinik IV, Department of Endocrinology, Klinikum der Universität München, Munich, Germany
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Jillian Bryce
Jillian Bryce
Office for Rare Conditions, University of Glasgow, Glasgow, UK
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Salma R Ali
Salma R Ali
Office for Rare Conditions, University of Glasgow, Glasgow, UK
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Roberta Giordano
Roberta Giordano
Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
Division of Endocrinology, Diabetes and Metabolism, Department of Medical Sciences, University of Turin, Turin, Italy
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Division of Endocrinology, Diabetes and Metabolism, Department of Medical Sciences, University of Turin, Turin, Italy
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Federico Baronio
Federico Baronio
Pediatric Unit, Department Hospital of Woman and Child, Endo-ERN Centre IT11, IRCSS AOU S.Orsola-Malpighi University Hospital, Bologna, Italy
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Irina Chifu
Irina Chifu
Division of Endocrinology and Diabetology, Department of Internal Medicine I, University Hospital of Wuerzburg, University of Wuerzburg, Wuerzburg, Germany
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Lea Tschaidse
Lea Tschaidse
Medizinische Klinik IV, Department of Endocrinology, Klinikum der Universität München, Munich, Germany
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Martine Cools
Martine Cools
Department of Paediatric Endocrinology, Ghent University Hospital, University of Ghent, Ghent, Belgium
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Erica LT van den Akker
Erica LT van den Akker
Department of Pediatrics, Division of Pediatric Endocrinology, Erasmus MC - Sophia Children’s Hospital, Erasmus University Medical Center, Rotterdam, The Netherlands
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Henrik Falhammar
Henrik Falhammar
Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
Department of Endocrinology, Karolinska University Hospital, Stockholm, Sweden
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Department of Endocrinology, Karolinska University Hospital, Stockholm, Sweden
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Natasha M Appelman-Dijkstra
Natasha M Appelman-Dijkstra
Department of Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, The Netherlands
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Luca Persani
Luca Persani
Department of Endocrine and Metabolic Diseases, Istituto Auxologico Italiano IRCCS, Milan, Italy
Department of Medical Biotechnology and Translational Medicine, University of Milan, Milan Italy
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Department of Medical Biotechnology and Translational Medicine, University of Milan, Milan Italy
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Guglielmo Beccuti
Guglielmo Beccuti
Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
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Ian L Ross
Ian L Ross
Division of Endocrinology, Department of Medicine, University of Cape Town, Cape Town, South Africa
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Simona Grozinsky-Glasberg
Simona Grozinsky-Glasberg
Neuroendocrine Tumor Unit, ENETS Center of Excellence, Department of Endocrinology and Metabolism, Hadassah Medical Organisation and Faculty of Medicine, the Hebrew University, Jerusalem, Israel
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Alberto M Pereira
Alberto M Pereira
Department of Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, The Netherlands
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Eystein S Husebye
Eystein S Husebye
Department of Clinical Science and KG Jebsen Center for Autoimmune Disorders, University of Bergen, Bergen, Norway
Department of Medicine, Haukeland University Hospital, Bergen, Norway
Department of Medicine, Karolinska Institutet, Stockholm, Sweden
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Department of Medicine, Haukeland University Hospital, Bergen, Norway
Department of Medicine, Karolinska Institutet, Stockholm, Sweden
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Stefanie Hahner
Stefanie Hahner
Division of Endocrinology and Diabetology, Department of Internal Medicine I, University Hospital of Wuerzburg, University of Wuerzburg, Wuerzburg, Germany
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S Faisal Ahmed
S Faisal Ahmed
Office for Rare Conditions, University of Glasgow, Glasgow, UK
Department of Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, The Netherlands
Developmental Endocrinology Research Group, University of Glasgow, Glasgow, United Kingdom
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Department of Medicine, Division of Endocrinology, Leiden University Medical Center, Leiden, The Netherlands
Developmental Endocrinology Research Group, University of Glasgow, Glasgow, United Kingdom
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Nicole Reisch
Nicole Reisch
Medizinische Klinik IV, Department of Endocrinology, Klinikum der Universität München, Munich, Germany
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insufficiency 34 23 Primary 25 20 Autoimmune 14 5 Congenital adrenal hyperplasia 9 10 Others 2 5 Secondary 5 2 Tertiary 4 1 Cushing’s syndrome 7 0 Pituitary 3
Sriharsha Gunna
Sriharsha Gunna
Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
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Mahaveer Singh
Mahaveer Singh
Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
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Rakesh Pandey
Rakesh Pandey
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
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Rungmei S K Marak
Rungmei S K Marak
Department of Microbiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
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Amita Aggarwal
Amita Aggarwal
Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
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Bibhuti Mohanta
Bibhuti Mohanta
Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
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Liping Yu
Liping Yu
Barbara Davis Centre for Diabetes, School of Medicine University of Colorado, Aurora, Colorado, USA
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Eesh Bhatia
Eesh Bhatia
Department of Endocrinology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
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Primary adrenal insufficiency (PAI) or Addison’s disease is an uncommon disorder resulting from deficiency of cortisol and aldosterone due to the destruction of adrenal cortices ( 1 , 2 ). Early diagnosis and prompt institution of treatment is
