Clinical Variability in Congenital Adrenal Hyperplasia: A Distinct Subgroup with A Low Glucocorticoid Dose Requirement
Authors' conclusion
Our findings highlight a subgroup of patients with 21-hydroxylase deficiency who achieve good control on low glucocorticoid doses from early childhood. The factors underlying this and the transient need for low doses in some remain unclear.
Study context
- Population
- Patients with salt-wasting congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiencypatients with CAH
- Participants
- 154 patients
- Exposure
- Low-dose glucocorticoid vs matched group on standard doses
- Low-dosen = 14
- Matchedn = 23reference
- Outcome
- Prevalence of low-dose glucocorticoid requirement (<10 mg/m²/day)
Methodological detailschart review, matched comparison group
- Chart review
- Matched comparison group
Main finding
As reported in the abstract
Low-dose
9%
14
Observational data: associations, not effects.
Further findings
Glucocorticoid dose
P < 0.001
Fludrocortisone dose
- Similar fludrocortisone doses
- 0.1 ± 0.05 mg
Weight, height, height velocity
- No differences in weight, height, or height velocity
Increase in glucocorticoid requirement above 10 mg/m²/day among low-dose patients
- Patients with increased requirement (at ages 10.3, 10.8, 8.5 years, after 6.3-9.8 mg/m²/day for 6.4-8.5 years)
- 3 of 14
- Remaining patients currently on 5.89-10 mg/m²/day, low-dose duration 0.48 to 8.65 years
- 11
Original publication
Clinical Variability in Congenital Adrenal Hyperplasia: A Distinct Subgroup with A Low Glucocorticoid Dose Requirement. Horm Res Paediatr. Published 23 June 2025. doi:10. 1159/ 000546883
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