ESPE Abstracts (2022) 95 P1-582

1Developmental Endocrinology Research Group, School of Medicine, Dentistry & Nursing, University of Glasgow, Glasgow, UK, Glasgow, United Kingdom; 2Office for Rare Conditions, Royal Hospital for Children & Queen Elizabeth University Hospital, Glasgow, United Kingdom; 3Lady Ridgeway Hospital, Colombo 00800, Sri Lanka, Colombo, Sri Lanka; 4Department of Paediatrics, Aarhus University Hospital, Aarhus DK-8200, Denmark, Aarhus, Denmark; 5Department Hospital of Woman and Child, Pediatric Unit, Center for Rare Endocrine Conditions (Endo-ERN), IRCCS - S.Orsola-Malpighi University Hospital, 40138 Bologna, Italy, Bologna, Italy; 6Department of Paediatrics, Technical University München, D-80804 Munich, Germany and and Department of Paediatrics, Klinikum Wels-Grieskirchen, A-4600 Wels, Austria, Wels, Austria; 7Department of Paediatric Endocrinology, Amalia Children’s Hospital, Radboud University Medical Centre, Nijmegen, the Netherlands, Nijmegen, Netherlands; 8University Hospital Ghent, Ghent University, 9000 Ghent, Belgium, Ghent, Belgium; 9Istanbul Faculty of Medicine, Department of Paediatrics, Paediatric Endocrinology Unit, Istanbul University, Çapa 34093, Istanbul, Turkey, Istanbul, Turkey; 10AOU Città della Salute- Regina Margherita Children's Hospital, Torino, Italy, Torino, Italy; 11Department of Pediatrics, Ain Shams University, 11566, Cairo, Egypt, Cairo, Egypt; 12Emma Children’s Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Pediatric Endocrinology, 1081HV Amsterdam, The Netherlands, Amsterdam, Netherlands; 13Pediatric Endocrinology, Diabetology and Metabolism, Department of Pediatrics and Department of Biomedical Research, Bern University Hospital Inselspital, University of Bern, 3010 Bern, Switzerland, Bern, Switzerland; 14Department of Endocrinology, William Harvey Research Institute, Barts and the London, School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK, London, United Kingdom; 15Marmara University, Department of Pediatric Endocrinology and Diabetes, 34899 Pendik, Istanbul, Turkey, Istanbul, Turkey; 16Baskent University Faculty of Medicine, Pediatric Endocrinology, Istanbul Hospital, Istanbul, Turkey; 17Department of Paediatrics, University of Cambridge, Cambridge CB2 2QQ, UK, Cambridge, United Kingdom; 18Department of Paediatrics, Medical University-Varna, UMHAT “Sv. Marina,” 9002 Varna, Bulgaria, Varna, Bulgaria; 19Birmingham Women’s & Children’s Hospital, Department for Endocrinology & Diabetes, Birmingham B4 6NH, UK, Birmingham, United Kingdom; 20Department of Endocrinology, University of Medicine and Pharmacy Craiova, Craiova 200349, Romania, Craiova, Romania; 21Unidade de Endocrinologia do Desenvolvimento, Laboratório de Hormônios e Genética Molecular/LIM42, Disciplina de Endocrinologia, Hospital Das Clinicas, Faculdade De Medicina, Universidade de Sao Paulo, São Paulo - SP, 05403-000, Brazil, Sao Paulo, Brazil; 22Department of Endocrinology, Mother and Child Health Care Institute of Serbia “Dr Vukan Čupić,” 11070 Belgrade, Serbia, Belgrade, Serbia; 23University of Magdeburg, 39106 Magdeburg, Germany, Magdeburg, Germany; 24Karolinska University Hospital, SE-171 77 Stockholm, Stockholm, Sweden; 25Wilhelmina Kinderziekenhuis, Division of Pediatric Endocrinology, 3584 EA Utrecht, Netherlands, Utrecht, Germany

Introduction: The reported occurrence and management of acute adrenal insufficiency (AI) – related adverse events (AE) vary widely between centres and may depend on available resources.

Aim: To use the data within the I-CAH Registry to understand the association between the occurrence and management of AI related AE, their management and the availability of local resources.

Methods: Of the 32 I-CAH centres that reported on the occurrence of SDE (, 24 completed a health care survey of local resources and clinical management with one point assigned for each resource. This included 16 centres from high income countries (HIC) and 8 from low/middle income countries (LMIC). Real world data from these 24 centres (420 children) were linked to the results of the health care survey from the centre.

Results: The median reported rate of sick day episodes (SDE, per patient year per centre) at HIC and LMIC centres was 0.75 (range, 0, 6) and 0.11 (0, 0.49), respectively, (P<0.05). Although, the availability of resources for management of AI – related AE was numerically greater at HIC centres vs LMIC centres with a median score of 5.5 (1,7) and 3.5 (2, 6), respectively, this did not reach statistical significance. The resources included written plans (100% of HIC vs 75% of LMIC), steroid emergency plans (87% of HIC vs 63% of LMIC, and family support events (38% of HIC vs 25% of LMIC). On the other hand, provision of contact details of medical and nursing team was reported more often in LMIC (75% of HIC vs 88% of LMIC). There was no relationship between the availability of resources and the rate of SDE in LMIC or HIC centres. The use of double dose hydrocortisone was reported more frequently in LMIC vs HIC centres (50% vs 31%). For management of adrenal crises, use of parenteral hydrocortisone was reported in 100% of HIC vs 63% of LMIC. On the other hand, the following were reported to be used more frequently in LMIC centres: prednisolone (LMIC:HIC - 38%:19%); glucagon (LMIC:HIC - 25%:13%); admission duration of 3 days (LMIC:HIC - 88%:25%).

Conclusions: Clear differences exist between the occurrence, prevention and management of acute AI–related AE in HIC and LMIC centres. The report of higher SDE rates in HIC than LMIC centres and the lack of a clear association at all centres between the level of resources available and the rate of SDE needs further study.

Volume 95

60th Annual ESPE (ESPE 2022)

Rome, Italy
15 Sep 2022 - 17 Sep 2022

European Society for Paediatric Endocrinology 

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