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.
Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022 / Tseretopoulou, Xanthippi; Ali, Salma R; Bryce, Jillian; Amin, Nadia; Atapattu, Navoda; Bachega, Tania A S S; Baronio, Federico; Ortolano, Rita; Birkebaek, Niels H; Bonfig, Walter; Cools, Martine; Davies, Justin H; Thomas, Tessy; de Vries, Liat; Elsedfy, Heba; Amr, Nermine H; Flueck, Christa E; Globa, Evgenia; Guran, Tulay; Yavas-Abali, Zehra; Guven, Ayla; Hannema, Sabine E; Iotova, Violeta; Konrad, Daniel; Lenherr-Taube, Nina; Krone, Nils P; Leka-Emiri, Sofia; Vlachopapadopoulou, Elpis; Lichiardopol, Corina; Marginean, Otilia; Markosyan, Renata; Neumann, Uta; Niedziela, Marek; Banaszak-Ziemska, Magdalena; Phan-Hug, Franziska; Poyrazoglu, Sukran; Probst-Scheidegger, Ursina; Randell, Tabitha; Russo, Gianni; Salerno, Mariacarolina; Seneviratne, Sumudu; Shnorhavorian, Margarett; Thankamony, Ajay; Tadokoro-Curraro, Rieko; van den Akker, Erica; van Eck, Judith; Vieites, Ana; Wasniewska, Malgorzata; Ahmed, S Faisal. - In: JOURNAL OF THE ENDOCRINE SOCIETY. - ISSN 2472-1972. - 8:10(2024). [10.1210/jendso/bvae145]
Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022
Salerno, Mariacarolina;
2024
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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
