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. 2024 Aug 20;8(10):bvae145.
doi: 10.1210/jendso/bvae145. eCollection 2024 Aug 27.

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

Xanthippi Tseretopoulou  1   2 , Salma R Ali  1   2 , Jillian Bryce  2 , Nadia Amin  3 , Navoda Atapattu  4 , Tania A S S Bachega  5 , Federico Baronio  6 , Rita Ortolano  6 , Niels H Birkebaek  7 , Walter Bonfig  8   9 , Martine Cools  10 , Justin H Davies  11 , Tessy Thomas  11 , Liat de Vries  12   13 , Heba Elsedfy  14 , Nermine H Amr  14 , Christa E Flueck  15   16 , Evgenia Globa  17 , Tulay Guran  18 , Zehra Yavas-Abali  18 , Ayla Guven  19 , Sabine E Hannema  20 , Violeta Iotova  21 , Daniel Konrad  22 , Nina Lenherr-Taube  22 , Nils P Krone  23 , Sofia Leka-Emiri  24 , Elpis Vlachopapadopoulou  24 , Corina Lichiardopol  25 , Otilia Marginean  26 , Renata Markosyan  27 , Uta Neumann  28 , Marek Niedziela  29 , Magdalena Banaszak-Ziemska  29 , Franziska Phan-Hug  30   31 , Sukran Poyrazoglu  32 , Ursina Probst-Scheidegger  33 , Tabitha Randell  34 , Gianni Russo  35 , Mariacarolina Salerno  36 , Sumudu Seneviratne  37 , Margarett Shnorhavorian  38 , Ajay Thankamony  39 , Rieko Tadokoro-Curraro  39 , Erica van den Akker  40 , Judith van Eck  40 , Ana Vieites  41 , Malgorzata Wasniewska  42 , S Faisal Ahmed  1   2
Affiliations

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

Xanthippi Tseretopoulou et al. J Endocr Soc. .

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.

Keywords: 21-hydroxylase deficiency; adrenal insufficiency; adverse events; benchmark; congenital adrenal hyperplasia; quality improvement; registry; sick day episodes.

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Figures

Figure 1.
Figure 1.
Violin plots showing the median adrenal insufficiency-related adverse events rate sick day episode (SDE), adrenal crises (AC) and hospitalization per patient-year in the 2019 cohort (covering visit data between 1984 and 2019) and the 2022 cohort (covering visit data between 2019 and 2022). *P = .01.
Figure 2.
Figure 2.
Funnel plots showing the sick day episode (SDE) benchmarks in 2019 and 2022. The continuous line indicates the overall mean SDE for the 2019 and 2022 cohort. Each center is represented by a number, which is the same if they participated in the 2019 and 2022 cohorts. The dotted lines indicate the 2 SD and 3 SD from the overall mean.
Figure 3.
Figure 3.
Change in reported sick day episode (SDE) rates in the 21 centers that participated in both 2019 and 2022 cohorts expressed as median SDE per patient-year per center. Reduction in SDE per patient year was noted in 13 centers, in 5 centers the SDE rate remained stable, and increased SDE rate was noted in 3 centers.
Figure 4.
Figure 4.
Trend of SDEs per patient year in yearly intervals during 2016-2022.
Figure 5.
Figure 5.
Median sick day episode (SDE) per patient-year by age bands for all phenotypes and for only the salt-wasting (SW) phenotype based on the data from the 2019 and 2022 cohorts, respectively. **P < .01 and *P < .05.
See this image and copyright information in PMC

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