ESPE2023 Poster Category 1 Adrenals and HPA Axis (40 abstracts)
Acute Adrenal Insufficiency Related Adverse Events In Children With Congenital Adrenal Hyperplasia (CAH): Changes During The Period 2019-2022 In I-CAH
Xanthippi Tseretopoulou 1,2 , Salma R Ali 1,2 , Jillian Bryce 2 , Nadia Amin 3 , Navoda Atapattu 4 , Tania Bachega 5 , Federico Baronio 6 , Niels H Birkebaek 7 , Walter Bonfig 8 , Justin H Davies 9 , Tessy Thomas 9 , Liat de Vries 10,11 , Heba Elsedfy 12 , Nermine Hussein Amr 12 , Christa E. Flueck 13 , Evgenia Globa 14 , Natalia Zelinska 14 , Tulay Guran 15 , Zehra Yavas Abali 15 , Ayla Guven 16 , Sabine E. Hannema 17 , Violeta Iotova 18 , Daniel Konrad 19 , Nils P. Krone 20 , Nina Lenherr-Taube 19 , Sofia Leka-Emiris 21 , Elpis Vlachopapadopoulou 21 , Corina R. Lichiardopol 22 , Otilia Marginean 23 , Renata L. Markosyan 24 , Marek Niedziela 25 , Magdalena Banaszak-Ziemska 25 , Uta Neumann 26 , Franziska Phan-Hug 27,28 , Sukran Polyrazoglu 29 , Ursina Probst 30 , Tabitha Randell 31 , Gianni Russo 32 , Mariacarolina Salerno 33 , Margarett Shnorhavorian 34 , Judith van Eck 35 , Ana Vieites 36 , Malgorzata Wasniewska 37 & Syed F Ahmed 1,2
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, UK, Glasgow, United Kingdom. 3Department of Paediatric Endocrinology, Leeds Teaching Hospitals NHS Trust, UK, Leeds, United Kingdom. 4Lady Ridgeway Hospital, Colombo, Sri Lanka, Colombo, Sri Lanka. 5Unidade 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, Brazil, Sao Paulo, Brazil. 6Department 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. 7Department of Paediatrics, Aarhus University Hospital, Aarhus N., Denmark, Aarhus, Denmark. 8Department of Paediatrics, Technical University München, D-80804 Munich, Germany and Department of Paediatrics, Klinikum Wels-Grieskirchen, A-4600 Wels, Austria, Wels, Austria. 9Department of Paediatric Endocrinology, University Hospital Southampton, UK, Southampton, United Kingdom. 10The Jesse Z and Sara Lea Shafer Institute for Endocrinology and Diabetes, Schneider Children’s Medical Center of Israel, Petach, Israel, Petah Tikva, Israel. 11Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel, Tel Aviv, Israel. 12Department of Pediatrics, Ain Shams University, 11566, Cairo, Egypt, Cairo, Egypt. 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. 14Ukrainian Research Centre of Endocrine Surgery, Endocrine Organs and Tissue Transplantation, MoH of Ukraine, Kyiv, Ukraine. 15Marmara University, Department of Pediatric Endocrinology and Diabetes, 34899 Pendik, Istanbul, Turkey, Istanbul, Turkey. 16Başkent University Faculty of Medicine, Istanbul Hospital Pediatric Endocrine, Istanbul, Turkey. 17Emma Children’s Hospital, Amsterdam UMC, Vrije Universiteit Amsterdam, Pediatric Endocrinology, 1081HV Amsterdam, The Netherlands, Amsterdam, Netherlands. 18Department of Paediatrics, Medical University-Varna, UMHAT “Sv. Marina,” 9002 Varna, Bulgaria, Varna, Bulgaria. 19Department of Endocrinology & Diabetology, University Children’s Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland. 20University of Sheffield, Sheffield, UK, Sheffield, United Kingdom. 21P&A Kyriakou Childrens’ Hospital, Athens, Greece, Athens, Greece. 22Department of Endocrinology, University of Medicine and Pharmacy Craiova, University Emergency Hospital, Craiova, Romania, Craiova, Romania. 23University of medicine and Pharmacy "Victor Babes", I st Pediatric Clinic, The research centre for child growth and development disorders – BELIVE, Clinical children emergency hospital "Louis Turcanu" Timisora, Romania, Timisora, Romania. 24epartment of Endocrinology, YSMU, Yerevan, Armenia, Yerevan, Armenia. 25Department of Pediatric Endocrinology and Rheumatology, Institute of Pediatrics, Poznan University of Medical Sciences, Poland, Poznan, Poland. 26Centre for Chronic Sick Children, Institute for Experimental Paediatric Endocrinology, Charité - Universitätsmedizin Berlin, Berlin, Germany, Berlin, Germany. 27Paediatric Endocrinology, EHC-Morges, Switzerland, Morges, Switzerland. 28Paediatric Endocrinology, Hospital Valais-Sion, Switzerland, Sion, Switzerland. 29Istanbul Faculty of Medicine, Department of Paediatrics, Paediatric Endocrinology Unit, Istanbul University, Çapa 34093, Istanbul, Turkey, Istanbul, Turkey. 30Kantonsspital Winterthur, Switzerland, Winterthur, Switzerland. 31Nottingham Children's Hospital, UK, Nottingham, United Kingdom. 32RCCS Ospedale San Raffaele, Milan Italy, Milan, Italy. 33Pediatric Endocrinology Unit- Department of Translational Medical Sciences, University Federico II Naples, Italy, Naples, Italy. 34University of Washington School of Medicine, Pediatric Urologist | Seattle Children's Hospital, Pediatric Urologist | Seattle Children's Hospital, USA, Seattle, USA. 35Sophia Children’s Hospital, Erasmus Medical Center, 3015 CN Rotterdam, The Netherlands; Leiden University Medical Center, 2300 RC Leiden, The Netherlands, Leiden, Netherlands. 36Centro de Investigaciones Endocrinológicas “Dr César Bergadá” (CEDIE), CONICET-FEI-División de Endocrinología, Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina, Buenos Aires, Argentina. 37Pediatric Unit, Department of Human Pathology of Adulthood and Childhood, University of Messina, Italy, Messina, Italy
Background: In 2019, the International CAH Registry (I-CAH) performed a benchmarking exercise of acute adrenal insufficiency related adverse events including adrenal crises (AC) and sick day episodes (SDE).
Methods: In 2022, I-CAH data on children aged <18 years at first visit with 21-hydroxylase deficiency CAH from 35 centres in 19 countries were analysed to examine the current occurrence of SDE and AC and compare it to the data that had been collected up to 2019 when 34 centres had participated in this exercise.
Results: In 2022, a total of 510 children with a median of 10 children (range 1, 58) per centre had 2,461 visits evaluated over a 3-yr period (2019-2022). The median patient age at the time of the visits was 8.3yrs (range 0, 20.6). The median duration of follow up per patient was 1.9yrs (0.1, 3.0) with a median of 2.4 visits (0.7, 20.6) per patient year. In 2022, of the 2,461 visits in 510 children, a total of 464 SDE were reported in 402 visits (16%) from 207 children (41%). Of the 464 SDE, reported management included an increase in oral glucocorticoids in 389 episodes (84%) and parenteral hydrocortisone in 68 episodes (15%); 280 episodes (60%) were self-managed in the community. The median SDE per patient per visit in 2019 and 2022 were 1 (0, 4) and 0 (0, 2), respectively. The median AC per patient per visit were 0 (0, 1) in both 2019 and 2022. The percentage of AC to total SDE in 2019 and 2022 were 4% and 6.5%, respectively. Infectious illness remained the most frequent precipitating event, reported in 69% (321 of 464 SDE) in 2022 and 72% (1,105 of 1,544 SDE) in 2019. Comparing the 19 centres that participated in both 2019 and 2022, the median SDE per patient year per centre in 2019 and 2022 was 0.4 (0, 6) and 0 (0, 2.5), respectively in 2022 (P=0.01). Of these 19 centres, 11 (58%) showed a reduction in SDE per patient year in 2022 compared to 2019 in 3 centres (16%) the SDE rate had increased and in 5 (26%) it remained unchanged.
Conclusions: The current study shows that the rate of reported SDE has fallen in 2022 compared to 2019. There is a need to continue widening participation whilst exploring the underlying factors that have led to a change in the rate of reported SDE.
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