Adrenal disease

Frequency of stress dosing and adrenal crisis in paediatric and adult patients with congenital adrenal hyperplasia: a prospective study

Eur J EndocrinolPublished Prospective observational study

Authors' conclusion

The AC incidence and frequency of SD in children and adults with CAH are high. In contrast to the paediatric cohort, the majority of SD in adults was not in accordance with the DGE recommendations, underlining the need for structured and repeated education of patients with particular focus on transition.

Study context

Population
Paediatric and adult patients with congenital adrenal hyperplasia (CAH) on life-long glucocorticoid replacement, and their parents
Participants
187 vs 38
Exposure
Adults vs children with CAH
  • Adultsn = 187
  • Childrenn = 38
Outcome
Adrenal crisis (AC) incidence
Methodological detailsData on adrenal crisis (AC) and SD collected via patient diary; medical records reviewed and patient interviews in case of AC
  • Data on AC and SD collected via patient diary; medical records reviewed and patient interviews in case of AC

Main finding

As reported in the abstract

AC incidence

Adults

8.4 per 100 patient years

Children

5.1 in 100 py

Observational data: associations, not effects.

Further findings

SD performed according to DGE recommendations

OutcomeAdultsChildrenP value
Stress dosing (SD) episodes195.4 SD episodes per 100 py169.7 per 100 py
CAHKAQ disease knowledge scoreHigher in children16.0 (unspecified 4.0)18.0 (unspecified 1.0)P = 0.001

Correlations in adults

SD frequency vs AC incidence
r = .235, P = .011
SD frequency vs CAH knowledge assessment questionnaire (CAHKAQ) score
r = .233, P = .014
AC incidence vs CAHKAQ
r = .193, P = .026

Original publication

Frequency of stress dosing and adrenal crisis in paediatric and adult patients with congenital adrenal hyperplasia: a prospective study. Eur J Endocrinol. Published 8 April 2024. doi:10.1093/ejendo/lvae023

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