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

Xanthippi Tseretopoulou; Salma R. Ali; Jillian Bryce; Nadia Amin; Navoda Atapattu; Tania A.S.S. Bachega; Federico Baronio; Rita Ortolano; Niels H. Birkebaek; Walter Bonfig; Martine Cools; Justin H. Davies; Tessy Thomas; Liat De Vries; Heba Elsedfy; Nermine H. Amr; Christa E. Flueck; Evgenia Globa; Tulay Guran; Zehra Yavas-Abali; Ayla Guven; Sabine E. Hannema; Violeta Iotova; Daniel Konrad; Nina Lenherr-Taube; Nils P. Krone; Sofia Leka-Emiri; Elpis Vlachopapadopoulou; Corina Lichiardopol; Otilia Marginean; Renata Markosyan; Uta Neumann; Marek Niedziela; Magdalena Banaszak-Ziemska; Franziska Phan-Hug; Sukran Poyrazoglu; Ursina Probst-Scheidegger; Tabitha Randell; Gianni Russo; Mariacarolina Salerno; Sumudu Seneviratne; Margarett Shnorhavorian; Ajay Thankamony; Rieko Tadokoro-Curraro; Erica Van Den Akker; Judith Van Eck; Ana Vieites; Malgorzata Wasniewska; S. Faisal Ahmed

doi:10.1210/jendso/bvae145

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

Xanthippi Tseretopoulou, Salma R. Ali, Jillian Bryce, Nadia Amin, Navoda Atapattu, Tania A.S.S. Bachega, Federico Baronio, Rita Ortolano, Niels H. Birkebaek, Walter Bonfig, Martine Cools, Justin H. Davies, Tessy Thomas, Liat De Vries, Heba Elsedfy, Nermine H. Amr, Christa E. Flueck, Evgenia Globa, Tulay Guran, Zehra Yavas-AbaliAyla Guven, Sabine E. Hannema, Violeta Iotova, Daniel Konrad, Nina Lenherr-Taube, Nils P. Krone, Sofia Leka-Emiri, Elpis Vlachopapadopoulou, Corina Lichiardopol, Otilia Marginean, Renata Markosyan, Uta Neumann, Marek Niedziela, Magdalena Banaszak-Ziemska, Franziska Phan-Hug, Sukran Poyrazoglu, Ursina Probst-Scheidegger, Tabitha Randell, Gianni Russo, Mariacarolina Salerno, Sumudu Seneviratne, Margarett Shnorhavorian, Ajay Thankamony, Rieko Tadokoro-Curraro, Erica Van Den Akker, Judith Van Eck, Ana Vieites, Malgorzata Wasniewska, S. Faisal Ahmed^*

^*Corresponding author for this work

Pediatrics

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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.

Original language	English
Article number	bvae145
Journal	Journal of the Endocrine Society
Volume	8
Issue number	10
DOIs	https://doi.org/10.1210/jendso/bvae145
Publication status	Published - Oct 2024

Bibliographical note

Publisher Copyright:
© 2024 The Author(s). Published by Oxford University Press on behalf of the Endocrine Society.

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Tseretopoulou, X., Ali, S. R., Bryce, J., Amin, N., Atapattu, N., Bachega, T. A. S. S., Baronio, F., Ortolano, R., Birkebaek, N. H., Bonfig, W., Cools, M., Davies, J. H., Thomas, T., De Vries, L., Elsedfy, H., Amr, N. H., Flueck, C. E., Globa, E., Guran, T., ... Ahmed, S. F. (2024). Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022. Journal of the Endocrine Society, 8(10), Article bvae145. https://doi.org/10.1210/jendso/bvae145

@article{7db2ae0d28c84394bc563cf6563b28cc,

title = "Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022",

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.",

author = "Xanthippi Tseretopoulou and Ali, {Salma R.} and Jillian Bryce and Nadia Amin and Navoda Atapattu and Bachega, {Tania A.S.S.} and Federico Baronio and Rita Ortolano and Birkebaek, {Niels H.} and Walter Bonfig and Martine Cools and Davies, {Justin H.} and Tessy Thomas and {De Vries}, Liat and Heba Elsedfy and Amr, {Nermine H.} and Flueck, {Christa E.} and Evgenia Globa and Tulay Guran and Zehra Yavas-Abali and Ayla Guven and Hannema, {Sabine E.} and Violeta Iotova and Daniel Konrad and Nina Lenherr-Taube and Krone, {Nils P.} and Sofia Leka-Emiri and Elpis Vlachopapadopoulou and Corina Lichiardopol and Otilia Marginean and Renata Markosyan and Uta Neumann and Marek Niedziela and Magdalena Banaszak-Ziemska and Franziska Phan-Hug and Sukran Poyrazoglu and Ursina Probst-Scheidegger and Tabitha Randell and Gianni Russo and Mariacarolina Salerno and Sumudu Seneviratne and Margarett Shnorhavorian and Ajay Thankamony and Rieko Tadokoro-Curraro and {Van Den Akker}, Erica and {Van Eck}, Judith and Ana Vieites and Malgorzata Wasniewska and Ahmed, {S. Faisal}",

note = "Publisher Copyright: {\textcopyright} 2024 The Author(s). Published by Oxford University Press on behalf of the Endocrine Society.",

year = "2024",

month = oct,

doi = "10.1210/jendso/bvae145",

language = "English",

volume = "8",

journal = "Journal of the Endocrine Society",

issn = "2472-1972",

publisher = "Endocrine Society",

number = "10",

}

Tseretopoulou, X, Ali, SR, Bryce, J, Amin, N, Atapattu, N, Bachega, TASS, Baronio, F, Ortolano, R, Birkebaek, NH, Bonfig, W, Cools, M, Davies, JH, Thomas, T, De Vries, L, Elsedfy, H, Amr, NH, Flueck, CE, Globa, E, Guran, T, Yavas-Abali, Z, Guven, A, Hannema, SE, Iotova, V, Konrad, D, Lenherr-Taube, N, Krone, NP, Leka-Emiri, S, Vlachopapadopoulou, E, Lichiardopol, C, Marginean, O, Markosyan, R, Neumann, U, Niedziela, M, Banaszak-Ziemska, M, Phan-Hug, F, Poyrazoglu, S, Probst-Scheidegger, U, Randell, T, Russo, G, Salerno, M, Seneviratne, S, Shnorhavorian, M, Thankamony, A, Tadokoro-Curraro, R, Van Den Akker, E , Van Eck, J, Vieites, A, Wasniewska, M & Ahmed, SF 2024, 'Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022', Journal of the Endocrine Society, vol. 8, no. 10, bvae145. https://doi.org/10.1210/jendso/bvae145

TY - JOUR

T1 - Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022

AU - Tseretopoulou, Xanthippi

AU - Ali, Salma R.

AU - Bryce, Jillian

AU - Amin, Nadia

AU - Atapattu, Navoda

AU - Bachega, Tania A.S.S.

AU - Baronio, Federico

AU - Ortolano, Rita

AU - Birkebaek, Niels H.

AU - Bonfig, Walter

AU - Cools, Martine

AU - Davies, Justin H.

AU - Thomas, Tessy

AU - De Vries, Liat

AU - Elsedfy, Heba

AU - Amr, Nermine H.

AU - Flueck, Christa E.

AU - Globa, Evgenia

AU - Guran, Tulay

AU - Yavas-Abali, Zehra

AU - Guven, Ayla

AU - Hannema, Sabine E.

AU - Iotova, Violeta

AU - Konrad, Daniel

AU - Lenherr-Taube, Nina

AU - Krone, Nils P.

AU - Leka-Emiri, Sofia

AU - Vlachopapadopoulou, Elpis

AU - Lichiardopol, Corina

AU - Marginean, Otilia

AU - Markosyan, Renata

AU - Neumann, Uta

AU - Niedziela, Marek

AU - Banaszak-Ziemska, Magdalena

AU - Phan-Hug, Franziska

AU - Poyrazoglu, Sukran

AU - Probst-Scheidegger, Ursina

AU - Randell, Tabitha

AU - Russo, Gianni

AU - Salerno, Mariacarolina

AU - Seneviratne, Sumudu

AU - Shnorhavorian, Margarett

AU - Thankamony, Ajay

AU - Tadokoro-Curraro, Rieko

AU - Van Den Akker, Erica

AU - Van Eck, Judith

AU - Vieites, Ana

AU - Wasniewska, Malgorzata

AU - Ahmed, S. Faisal

PY - 2024/10

Y1 - 2024/10

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=85203970625&partnerID=8YFLogxK

U2 - 10.1210/jendso/bvae145

DO - 10.1210/jendso/bvae145

M3 - Article

C2 - 39258010

AN - SCOPUS:85203970625

SN - 2472-1972

VL - 8

JO - Journal of the Endocrine Society

JF - Journal of the Endocrine Society

IS - 10

M1 - bvae145

ER -

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

Abstract

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