ArticleThe Journal of clinical endocrinology and metabolism2021
Real-World Estimates of Adrenal Insufficiency-Related Adverse Events in Children With Congenital Adrenal Hyperplasia.
Article in The Journal of clinical endocrinology and metabolism, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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Who cites it
20 citing papers in PubMed, 36 citations in OpenAlex.
- Glucocorticoid reduction after starting crinecerfont in pediatric patients with classic congenital adrenal hyperplasia: practical perspectives.The Journal of clinical endocrinology and metabolism · 2026Review
- Integrated mapping resolves pathogenicity of 11Journal of the Endocrine Society · 2026Article
- Adrenal Crisis Induced-seizures: A Case Report and Literature Review.Journal of community hospital internal medicine perspectives · 2026Article
- Approach to the Child and Adolescent With Adrenal Insufficiency.The Journal of clinical endocrinology and metabolism · 2025Review
- Cardiometabolic Aspects of Congenital Adrenal Hyperplasia.Endocrine reviews · 2025Review
- Patient and caregiver experiences with hydrocortisone injections in adrenal crisis: a mixed-methods cross-sectional study.Frontiers in endocrinology · 2025Observational
- Temporal Trends in Acute Adrenal Insufficiency Events in Children With Congenital Adrenal Hyperplasia During 2019-2022.Journal of the Endocrine Society · 2024Article
- Diagnosis and management of non-CAH 46,XX disorders/differences in sex development.Frontiers in endocrinology · 2024Review
- Interpretation of Steroid Biomarkers in 21-Hydroxylase Deficiency and Their Use in Disease Management.The Journal of clinical endocrinology and metabolism · 2023Review
- Adrenal insufficiency management in the pediatric emergency setting and risk factors for adrenal crisis development.Italian journal of pediatrics · 2023Observational
- Autoimmune Polyendocrine Syndromes in the Pediatric Age.Children (Basel, Switzerland) · 2023Review
- Cohort profile: pathways to care among people with disorders of sex development (DSD).BMJ open · 2022Article
- Article
- Management challenges and therapeutic advances in congenital adrenal hyperplasia.Nature reviews. Endocrinology · 2022Review
- Congenital Adrenal Hyperplasia-Current Insights in Pathophysiology, Diagnostics, and Management.Endocrine reviews · 2022Review
- [Emergency card, emergency medication, and information leaflet for the prevention and treatment of adrenal crisis (Addison crisis): an Austrian consensus document].Journal fur klinische Endokrinologie und Stoffwechsel · 2022Article
- Characterization of Mutations Causing CYP21A2 Deficiency in Brazilian and Portuguese Populations.International journal of molecular sciences · 2021Article
- Salivary Profiles of 11-oxygenated Androgens Follow a Diurnal Rhythm in Patients With Congenital Adrenal Hyperplasia.The Journal of clinical endocrinology and metabolism · 2021Article
- Disorders of Sex Development of Adrenal Origin.Frontiers in endocrinology · 2021Review
- Acute Illness and Death in Children With Adrenal Insufficiency.Frontiers in endocrinology · 2021Article
Corrections and comments
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Authors and funding
47 authors at 20 institutions in 19 countries.
Funding
Abstract
backgroundAlthough 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.
methodsData 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.
resultsA 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.
conclusionsThe 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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