ESPE2019 Poster Category 1 Adrenals and HPA Axis (1) (12 abstracts)
Development of an International Benchmark for Sick Day Episodes as a Core Clinical Outcome in People with Congenital Adrenal Hyperplasia
Salma Ali 1 , Eleni Daniel 2 , Jillian Bryce 1 , Adalia Ikiroma 1 , James Lewsey 3 , Richard Ross 2 , Ruth Krone 4 , Carlo Acerini 5 , Nils Krone 6 , Urmi Das 7 , Jeremy Tomlinson 8 , Marta Korbonits 9 , Claire Higham 10 , Feyza Darendeliler 11 , Tulay Guran 12 , Ayla Guven 13 , Navoda Attapatu 14 , Tatjana Milenkovic 15 , Corina Raducanu-Lichiardopol 16 , Sabine Hannema 17 , Hedi Claahsen 18 , Martijn Finken 19 , Federico Baronio 20 , Antonio Balsamo 20 , Silvia Einaudi 21 , Liat de Vries 22 , Andrea Luczay 23 , Uta Neumann 24 , Oliver Blankenstein 24 , Klaus Mohnike 25 , Walter Bonfig 26 , Heba Elsedfy 27 , Niels Birkebaek 28 , Violeta Iotova 29 , Tania Bachega 30 , Berenice Mendonca 30 , Martine Cools 31 , Eduardo Correa Costa 32 , Guilherme Guaragna-Filho 33 , Rodolfo Rey 34 & S. Faisal Ahmed 1
1Developmental Endocrinology Research Group, School of Medicine, Dentistry & Nursing, University of Glasgow, Glasgow, United Kingdom. 2Department of Oncology and Metabolism, University of Sheffield,, Sheffield, United Kingdom. 3Health Economics and Health Technology Assessment, Institute of Health and Wellbeing, University of Glasgow, Glasgow, United Kingdom. 4Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, United Kingdom. 5Department of Paediatrics, University of Cambridge, Cambridge, United Kingdom. 6University of Sheffield, Sheffield, United Kingdom. 7Alder Hey Childrens Hospital, Liverpool, United Kingdom. 8Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism (OCDEM) and NIHR Oxford Biomedical Research Centre, Churchill Hospital, University of Oxford, Oxford, United Kingdom. 9Centre for Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, United Kingdom. 10Department of Endocrinology, Christie Hospital Nhs Foundation Trust, Manchester, University Of Manchester, Manchester Academic Health Science Centre, Manchester, United Kingdom. 11Istanbul Faculty of Medicine, Department of Pediatrics, Pediatric Endocrinology Unit, Istanbul University, Istanbul, Turkey. 12Marmara University, Department of Pediatric Endocrinology and Diabetes, Istanbul, Turkey. 13Istanbul Medeniyet University, Goztepe Education and Research Hospital, Istanbul, Turkey. 14The Central Hospital, Columbo, Sri Lanka. 15University of Belgrade, Belgrade, Serbia. 16University of Medicine and Pharmacy, Craiova, Romania. 17Leiden University Medical Centre, Leiden, Netherlands. 18Radboudumc Amalia Children's Hospital, Nijmegen, Netherlands. 19VU University Medical Center, Department of Pediatrics, Subdivision of Endocrinology, Amsterdam, Netherlands. 20Department of Woman, Child and Urologic Diseases, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy. 21Torino - Regina Margherita Children's Hospital, Torino, Italy. 22The Jesse Z and Sara Lea Shafer Institute for Endocrinology And Diabetes, National Center For Childhood Diabetes, Schneider Children's Medical Center Of Israel, Petah Tikva, Israel. 23Ist Department of Paediatrics, Semmelweis University, Budapest, Hungary. 24Charité Universitätsmedizin, Berlin, Germany. 25Otto-von-Guericke Universität Magdeburg, Magdeburg, Germany. 26Department of Pediatrics, Klinikum Wels-Grieskirchen, Wels, Austria; Department of Pediatrics, Technical University München, Munich, Germany. 27Ain Shams University, Cairo, Egypt. 28Dept of Pediatrics, Aarhus University Hospital, Aarhus, Denmark. 29; Clinic Of Paediatric Endocrinology - Umhat 'St. Marina', Medical University of Varna, Varna, Bulgaria. 30University of Sao Paulo, Sao Paulo, Brazil. 31Department of Paediatric Endocrinology, Ghent University Hospital, Department of Internal Medicine and Paediatrics, Ghent, Belgium. 32Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil. 33Catholic University, Porto Alegre, Brazil. 34Centro de Investigaciones Endocrinológicas "Dr. César Bergadá" (CEDIE) CONICET – FEI – División de Endocrinología, Hospital de Niños Ricardo Gutiérrez Gallo 1330, Buenos Aires, Argentina
Background: Congenital adrenal hyperplasia (CAH) is a rare condition characterised by adrenal insufficiency and a life-long risk of adrenal crises. There is a paucity of information on the epidemiology of acute adverse events in this population.
Objective: To investigate the frequency, aetiology and consequences of acute adverse events attributed to adrenal insufficiency in CAH.
Methods: A longitudinal analysis of patients with CAH in the International Congenital Adrenal Hyperplasia Registry (I-CAH registry, www.i-cah.org) which collects information on acute adverse events including sick day episodes and adrenal crises.
Results: 509 patients (n= 478, 21-OHD) from 31 centres in 16 countries and a total of 3880 visits were evaluated. 261 patients (n=255, 21-OHD) had one or more sick day episodes (684 visits); of these, 215 (82%) were less than 18 years of age. 1034 sick day episodes were recorded in total, with 920 (89%) episodes recorded in those less than 18 years of age. The overall median number of sick day episodes for all centres per patient year was 3.0 for children (IQR 1.7-4.7) and 3.9 for adults (IQR 1.8-10.2) (P=0.26). The median duration of sick day episodes was 3 days (IQR 2.0-5.0) and 2 days (IQR 1.0-3.0) in children and adults respectively (P<0.05). During childhood, younger age and low hydrocortisone dose (mg/m2/day) were associated with a greater number of sick day episodes (P<0.01). Female sex was associated with higher rates of admission amongst both children and adults (P<0.01). Infectious illness was the most frequent event causing illness episodes and adrenal crises in both children (66%) and adults (23%). An adrenal crisis was reported in 37 (4%, 37/920) and 34 (30%, 34/114) sick day episodes amongst children and adults, respectively (P<0.05) and all adults required hospital admission.
Conclusions: The real world data within the I-CAH registry are a valuable resource for studying a core clinical outcome that can be used as a benchmark for improving clinical care. Further work needs to be undertaken to understand the determinants of the observed variations in the occurrence of sick day episodes.
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