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A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During
Xanthippi Tseretopoulou1,2, Salma R Ali1,2, Jillian Bryce2
1Developmental Endocrinology Research Group, School of Medicine, Dentistry & Nursing, University of Glasgow, Glasgow G51 4TF, UK.
Insights
Targeted monitoring in congenital adrenal hyperplasia (CAH) showed a decrease in sick day episodes (SDEs) and hospitalizations. These findings suggest the I-CAH Registry can track clinical benchmarks, but require careful interpretation and long-term observation.
Area of Science:
- Pediatric Endocrinology
- Rare Disease Research
- Clinical Trial Monitoring
Background:
- Congenital adrenal hyperplasia (CAH) management involves monitoring for acute adrenal insufficiency (AI) related adverse events (AE), including sick day episodes (SDEs) and hospitalizations.
- The impact of targeted monitoring on the occurrence of these AI-related AEs in CAH patients remains unclear.
Purpose of the Study:
- To investigate temporal trends of AI-related adverse events (AE) within the I-CAH Registry.
- To assess whether targeted monitoring is associated with changes in SDEs and hospitalization rates in children with CAH.
Main Methods:
- Data from children under 18 with 21-hydroxylase deficiency CAH in the I-CAH Registry were collected in 2022 and compared to data from 2019.
- A total of 513 children across 38 centers in 21 countries were evaluated over a 3-year period (2019-2022).
- Statistical analysis compared SDE rates and hospitalization rates due to SDEs between the 2019 and 2022 cohorts, adjusting for relevant factors.
Main Results:
- The median SDE rate per patient year decreased significantly from 0.3 in 2019 to 0 in 2022 (P = .01), even after adjustment.
- Hospitalization rates due to SDEs also decreased, with 22% of SDEs leading to hospitalization in 2022 compared to 27% in 2019 (P = .02).
- Of the 38 centers, 62% showed a reduction in SDE rates between the two periods.
Conclusions:
- The I-CAH Registry serves as a valuable tool for targeted monitoring of critical clinical benchmarks in CAH.
- While a reduction in SDEs and hospitalizations was observed, these changes necessitate careful interpretation and continued long-term monitoring.
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.
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