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Real-World Estimates of Adrenal Insufficiency-Related Adverse Events in Children With Congenital Adrenal Hyperplasia
Salma R Ali1,2, Jillian Bryce2, Houra Haghpanahan3
1Developmental Endocrinology Research Group, School of Medicine, Dentistry & Nursing, University of Glasgow, Glasgow, UK.
Insights
Congenital adrenal hyperplasia (CAH) patients experience sick day episodes (SDE), but their link to adrenal crises (AC) needs clarification. Infectious illness is a common trigger for both SDE and AC in children with CAH.
Area of Science:
- Endocrinology
- Pediatric Health
- Rare Diseases
Background:
- Congenital adrenal hyperplasia (CAH) is linked to adrenal crises (AC).
- The association between CAH and patient/clinician-reported sick day episodes (SDE) is less understood.
- Understanding SDE is crucial for managing CAH, particularly in children.
Purpose of the Study:
- To investigate the clinical factors associated with SDE and AC in children with classic 21-hydroxylase deficiency CAH.
- To analyze data from the International CAH Registry to compare SDE and AC occurrences.
- To examine differences in SDE and AC between low/middle-income countries (LMIC) and high-income countries (HIC).
Main Methods:
- Retrospective analysis of data from 518 children with classic 21-hydroxylase deficiency CAH.
- Data collected from 34 centers across 18 countries via the International CAH Registry.
- Evaluation of 5388 visits over 2300 patient-years, focusing on SDE and AC events.
Main Results:
- Infectious illness was the most frequent precipitant for both SDE (72%) and AC (47%).
- The median SDE per patient-year was significantly higher in LMIC (0.75) compared to HIC (0.11) (P < 0.001).
- No significant difference in AC rates was observed between LMIC and HIC.
Conclusions:
- Real-world data from the I-CAH Registry reveal variability in adverse events related to adrenal insufficiency.
- Further research is needed to standardize the definition of SDE in CAH care.
- Improved definition and understanding of SDE can enhance clinical benchmarks for CAH management.
Background:
Although congenital adrenal hyperplasia (CAH) is known to be associated with adrenal crises (AC), its association with patient- or clinician-reported sick day episodes (SDE) is less clear.
Methods:
Data on children with classic 21-hydroxylase deficiency CAH from 34 centers in 18 countries, of which 7 were Low or Middle Income Countries (LMIC) and 11 were High Income (HIC), were collected from the International CAH Registry and analyzed to examine the clinical factors associated with SDE and AC.
Results:
A total of 518 children-with a median of 11 children (range 1, 53) per center-had 5388 visits evaluated over a total of 2300 patient-years. The median number of AC and SDE per patient-year per center was 0 (0, 3) and 0.4 (0.0, 13.3), respectively. Of the 1544 SDE, an AC was reported in 62 (4%), with no fatalities. Infectious illness was the most frequent precipitating event, reported in 1105 (72%) and 29 (47%) of SDE and AC, respectively. On comparing cases from LMIC and HIC, the median SDE per patient-year was 0.75 (0, 13.3) vs 0.11 (0, 12.0) (P < 0.001), respectively, and the median AC per patient-year was 0 (0, 2.2) vs 0 (0, 3.0) (P = 0.43), respectively.
Conclusions:
The real-world data that are collected within the I-CAH Registry show wide variability in the reported occurrence of adrenal insufficiency-related adverse events. As these data become increasingly used as a clinical benchmark in CAH care, there is a need for further research to improve and standardize the definition of SDE.
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