Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022-Reference-Cited by-同舟云学术

Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022

Published:2024-08-20 Issue:10 Volume:8 Page:
ISSN:2472-1972
Container-title:Journal of the Endocrine Society
language:en
Short-container-title:

Author:

Tseretopoulou Xanthippi¹²^ORCID,Ali Salma R¹²,Bryce Jillian²,Amin Nadia³,Atapattu Navoda⁴^ORCID,Bachega Tania A S S⁵^ORCID,Baronio Federico⁶,Ortolano Rita⁶,Birkebaek Niels H⁷,Bonfig Walter⁸⁹,Cools Martine¹⁰,Davies Justin H¹¹^ORCID,Thomas Tessy¹¹,de Vries Liat¹²¹³,Elsedfy Heba¹⁴,Amr Nermine H¹⁴,Flueck Christa E¹⁵¹⁶,Globa Evgenia¹⁷,Guran Tulay¹⁸,Yavas-Abali Zehra¹⁸,Guven Ayla¹⁹,Hannema Sabine E²⁰,Iotova Violeta²¹,Konrad Daniel²²^ORCID,Lenherr-Taube Nina²²,Krone Nils P²³^ORCID,Leka-Emiri Sofia²⁴,Vlachopapadopoulou Elpis²⁴^ORCID,Lichiardopol Corina²⁵,Marginean Otilia²⁶,Markosyan Renata²⁷,Neumann Uta²⁸^ORCID,Niedziela Marek²⁹,Banaszak-Ziemska Magdalena²⁹,Phan-Hug Franziska³⁰³¹,Poyrazoglu Sukran³²,Probst-Scheidegger Ursina³³,Randell Tabitha³⁴,Russo Gianni³⁵,Salerno Mariacarolina³⁶^ORCID,Seneviratne Sumudu³⁷,Shnorhavorian Margarett³⁸,Thankamony Ajay³⁹,Tadokoro-Curraro Rieko³⁹,van den Akker Erica⁴⁰,van Eck Judith⁴⁰,Vieites Ana⁴¹,Wasniewska Malgorzata⁴²,Ahmed S Faisal¹²^ORCID

Affiliation:

1. Developmental Endocrinology Research Group, School of Medicine, Dentistry & Nursing, University of Glasgow , Glasgow G51 4TF , UK

2. Office for Rare Conditions, Royal Hospital for Children & Queen Elizabeth University Hospital , Glasgow G51 4TF , UK

3. Department of Paediatric Endocrinology, Leeds Teaching Hospitals NHS Trust , Leeds LS1 3EX , UK

4. Department of Paediatric Endocrinology, Lady Ridgeway Hospital , Colombo 00800 , Sri Lanka

5. Unidade 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, 05508-090 , Brazil

6. Department Hospital of Woman and Child, Pediatric Unit, IRCCS—Azienda Ospedaliero-Universitaria di Bologna , 40138 Bologna , Italy

7. Department of Paediatrics, Aarhus University Hospital , Aarhus DK-8200 , Denmark

8. Department of Paediatrics, Technical University München , D-80804 Munich , Germany

9. Department of Paediatrics, Klinikum Wels-Grieskirchen , A-4600 Wels , Austria

10. Department of Paediatric Endocrinology, University Hospital Ghent, Ghent University , 9000 Ghent , Belgium

11. Department of Paediatric Endocrinology, University Hospital Southampton , Southampton SO16 6YD , UK

12. The Jesse and Sara Lea Shafer Institute of Endocrinology and Diabetes, Schneider Children's Medical Center of Israel , Petah Tikvah 4920235 , Israel

13. Faculty of Medical&Health Sciences, Tel Aviv University , Tel Aviv 6997801 , Israel

14. Department of Pediatrics, Ain Shams University , 11566 Cairo , Egypt

15. Pediatric Endocrinology, Diabetology and Metabolism, Department of Pediatrics, Bern University Hospital Inselspital, University of Bern , 3010 Bern , Switzerland

16. Pediatric Endocrinology, Diabetology and Metabolism, Department of BioMedical Research, Bern University Hospital Inselspital, University of Bern , 3010 Bern , Switzerland

17. Ukrainian Scientific and Practical Center of Endocrine Surgery, Transplantation of Endocrine Organs and Tissues of MOH of Ukraine , Kyiv 01021 , Ukraine

18. Department of Pediatric Endocrinology and Diabetes, Marmara University , 34899 Pendik, Istanbul , Turkey

19. Department of Paediatric Endocrinology, Baskent University Medical Faculty, Istanbul Hospital , 06790 Istanbul , Turkey

20. Department of Pediatric Endocrinology, Amsterdam UMC location Vrije Universiteit Amsterdam , 1007 MB Amsterdam , The Netherlands

21. Department of Paediatrics, Medical University-Varna, UMHAT “Sv. Marina,” 9002 Varna , Bulgaria

22. Department of Endocrinology and Diabetology, University Children's Hospital Zurich, University of Zurich , 8032 Zurich , Switzerland

23. Department of Oncology and Metabolism, University of Sheffield , Sheffield S10 2RX , UK

24. Department of Endocrinology-Growth and Development, “P&A Kyriakou” Children's Hospital , Athens 115 27 , Greece

25. Department of Endocrinology, University of Medicine and Pharmacy Craiova, University Emergency Hospital , Craiova 200349 , Romania

26. Department of Paediatrics, University of medicine and Pharmacy “Victor Babes”, Clinical children emergency hospital “Louis Turcanu” Timisora , Timisora 300011 , Romania

27. Department of Endocrinology, YSMU , Yerevan 0025 , Armenia

28. Department of Paediatric Endocrinology and Diabetology, Centre for Chronic Sick Children, Institute for Experimental Paediatric Endocrinology, Charité-Universitätsmedizin Berlin , 10117 Berlin , Germany

29. Department of Pediatric Endocrinology and Rheumatology, Institute of Pediatrics, Poznan University of Medical Sciences , 61 701 Poznan , Poland

30. Paediatric Endocrinology, EHC-Morges , 1110 Morges , Switzerland

31. Paediatric Endocrinology, Hospital Valais-Sion , 1950 Sion , Switzerland

32. Istanbul Faculty of Medicine, Department of Paediatrics, Paediatric Endocrinology Unit, Istanbul University , 34093 Çapa, Istanbul , Turkey

33. Department of Paediatric Endocrinology, Kantonsspital Winterthur , 8401 Zurich , Switzerland

34. Department of Paediatric Endocrinology, Nottingham Children's Hospital , Nottingham NG7 2UH , UK

35. Department of Paediatrics, Endocrine Unit, RCCS Ospedale San Raffaele , 20132 Milan , Italy

36. Department of Pediatrics, Unit of Immunology, Federico II University , 80131 Naples , Italy

37. Department of Paediatrics, Faculty of Medicine, University of Colombo , Colombo 00700 , Sri Lanka

38. Department of Urology, University of Washington School of Medicine, Seattle Children's Hospital , Seattle, WA 98105 , USA

39. Department of Paediatrics,University of Cambridge , Cambridge CB2 2QQ , UK

40. Division of Pediatric Endocrinology, Department of Pediatrics, Erasmus MC-Sophia Children's Hospital , 3015 CN Rotterdam , The Netherlands

41. Centro de Investigaciones Endocrinológicas, División de Endocrinología, Hospital de Niños Ricardo Gutiérrez , 1425 Buenos Aires , Argentina

42. Pediatric Unit, Department of Human Pathology of Adulthood and Childhood, University of Messina , 98166 Messina , Italy

Abstract

Abstract Background It is unclear whether targeted monitoring of acute adrenal insufficiency (AI) related adverse events (AE) such as sick day episodes (SDEs) and hospitalization rate in congenital adrenal hyperplasia (CAH) is associated with a change in the occurrence of these events. Aim Study temporal trends of AI related AE in the I-CAH Registry. Methods In 2022, data on the occurrence of AI-related AE in children aged <18 years with 21-hydroxylase deficiency CAH were compared to data collected in 2019. Results In 2022, a total of 513 children from 38 centers in 21 countries with a median of 8 children (range 1-58) per center had 2470 visits evaluated over a 3-year period (2019-2022). The median SDE per patient year in 2022 was 0 (0-2.5) compared to 0.3 (0-6) in 2019 (P = .01). Despite adjustment for age, CAH phenotype and duration of study period, a difference in SDE rate was still apparent between the 2 cohorts. Of the 38 centers in the 2022 cohort, 21 had also participated in 2019 and a reduction in SDE rate was noted in 13 (62%), an increase was noted in 3 (14%), and in 5 (24%) the rate remained the same. Of the 474 SDEs reported in the 2022 cohort, 103 (22%) led to hospitalization compared to 299 of 1099 SDEs (27%) in the 2019 cohort (P = .02). Conclusion The I-CAH Registry can be used for targeted monitoring of important clinical benchmarks in CAH. However, changes in reported benchmarks need careful interpretation and longer-term monitoring.

Funder

Medical Research Council United Kingdom

Seventh Framework Program European Union

European Society for Paediatric Endocrinology United Kingdom

Publisher

The Endocrine Society

Link

https://academic.oup.com/jes/advance-article-pdf/doi/10.1210/jendso/bvae145/58859923/bvae145.pdf

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