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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
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
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
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
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
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
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
Hiren Patt
Hiren Patt
Department of Endocrinology, Seth G.S. Medical College & KEM Hospital, Mumbai, Maharashtra, India
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Katrin Koehler
Katrin Koehler
Department of Paediatrics, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
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Sailesh Lodha
Sailesh Lodha
Eternal Hospital, Jaipur, Rajasthan, 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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Chaitanya Yerawar
Chaitanya Yerawar
Department of Endocrinology, Seth G.S. Medical College & KEM Hospital, Mumbai, Maharashtra, India
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Angela Huebner
Angela Huebner
Department of Paediatrics, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
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Kunal Thakkar
Kunal Thakkar
Department of Endocrinology, Seth G.S. Medical College & KEM Hospital, Mumbai, Maharashtra, India
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Sneha Arya
Sneha Arya
Department of Endocrinology, Seth G.S. Medical College & KEM Hospital, Mumbai, Maharashtra, India
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Sandhya Nair
Sandhya Nair
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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Hosahithlu Ganesh
Hosahithlu Ganesh
Department of Endocrinology, AJ Institute of Medical sciences, Mangalore, India
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Vijaya Sarathi
Vijaya Sarathi
Department of Endocrinology, Vydehi Institute of Medical Sciences and Research Center, Bengaluru, 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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Tushar Bandgar
Tushar Bandgar
Department of Endocrinology, Seth G.S. Medical College & KEM Hospital, Mumbai, Maharashtra, India
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Nalini Shah
Nalini Shah
Department of Endocrinology, Seth G.S. Medical College & KEM Hospital, Mumbai, Maharashtra, India
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. ( doi:10.1073/pnas.0505598103 ) 10.1073/pnas.0505598103 7 Bornstein SR Allolio B Arlt W Barthel A Don-Wauchope A Hammer GD Husebye ES Merke DP Murad MH Stratakis CA Diagnosis and treatment of primary adrenal insufficiency: an Endocrine Society
Marcus Quinkler
Marcus Quinkler
Endocrinology in Charlottenburg, Berlin, Germany
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Bertil Ekman
Bertil Ekman
Departments of Endocrinology and Medical and Health Sciences, Linköping University, Linköping, Sweden
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Claudio Marelli
Claudio Marelli
Shire International GmbH, Zug, Switzerland
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Sharif Uddin
Sharif Uddin
Shire, Lexington, Massachusetts, USA
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Pierre Zelissen
Pierre Zelissen
Department of Internal Medicine and Endocrinology, University Medical Center Utrecht, Utrecht, the Netherlands
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Robert D Murray
Robert D Murray
Department of Endocrinology, Leeds Teaching Hospitals NHS Trust, St James’s University Hospital, Leeds, UK
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on behalf of the EU-AIR Investigators
on behalf of the EU-AIR Investigators
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-AIR, European Adrenal Insufficiency Registry; PAI, primary adrenal insufficiency; SAI, secondary adrenal insufficiency; s.d. , standard deviation. After predefining the matching cohorts, we performed a 1:3 matching of patients on prednisolone
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
