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Incidence and Risk Factors for Adrenal Crisis in Pediatric-onset Adrenal Insufficiency: A Prospective Study
Mayumi Hosokawa1,2,3, Yosuke Ichihashi1,2, Yasunori Sato4
1Japanese Multicenter Study for Adrenal Crisis (JMSAC), Tokyo 160-8582, Japan.
Insights
Pediatric patients with adrenal insufficiency (AI) face a significant risk of adrenal crisis (AC). Younger age and frequent infections are key risk factors for developing AC in children with AI.
Area of Science:
- Pediatric Endocrinology
- Adrenal Gland Disorders
- Clinical Research
Background:
- Adrenal crisis (AC) is a severe complication in patients with adrenal insufficiency (AI).
- Limited prospective data exists on AC in pediatric primary and secondary AI.
Purpose of the Study:
- To investigate the incidence and identify risk factors for adrenal crisis (AC) in children with AI.
- To provide crucial data for managing pediatric AI patients and preventing AC.
Main Methods:
- A multicenter, prospective cohort study in Japan involving 349 children (age ≤15 years) with AI.
- Incidence calculated per person-year (PY); risk factors analyzed using Poisson regression.
Main Results:
- The study recorded 41 AC events in 31 patients over 961 PY, yielding an incidence of 4.27 per 100 PY.
- Younger age at enrollment (RR 0.93) and increased infections (RR 1.17) were significant risk factors for AC.
- Female sex, primary AI, and hydrocortisone dosage were not significant risk factors.
Conclusions:
- A considerable number of pediatric-onset AI patients experience adrenal crisis (AC).
- Younger age and a higher frequency of infections independently increase AC risk in this population.
Context:
Adrenal crisis (AC) is a life-threatening complication that occurs during follow-up of patients with adrenal insufficiency (AI). No prospective study has thoroughly investigated AC in children with primary and secondary AI.
Objective:
This work aimed to determine the incidence and risk factors for AC in patients with pediatric-onset AI.
Methods:
This multicenter, prospective cohort study conducted in Japan enrolled patients diagnosed with AI at age ≤15 years. The incidence of AC was calculated as events per person-year (PY), and risk factors for AC were assessed using Poisson regression multivariable analysis.
Results:
The study population comprised 349 patients (164 male, 185 female) with a total follow-up of 961 PY. The median age at enrollment was 14.3 years (interquartile range [IQR] 8.5-21.2 years), and the median follow-up was 2.8 years (IQR 2.2-3.3 years). Of these patients, 213 (61%) had primary AI and 136 (39%) had secondary AI. Forty-one AC events occurred in 31 patients during the study period. The calculated incidence of AC was 4.27 per 100 PY (95% CI, 3.15-5.75). Poisson regression analysis identified younger age at enrollment (relative risk [RR] 0.93; 95% CI, 0.89-0.97) and increased number of infections (RR 1.17; 95% CI, 1.07-1.27) as significant risk factors. Female sex (RR 0.99; 95% CI, 0.53-1.86), primary AI (RR 0.65; 95% CI, 0.30-1.41), or equivalent dosage of hydrocortisone per square meter of body area (RR 1.02; 95% CI, 0.96-1.08) was not a significant risk factor.
Conclusion:
A substantial proportion of patients with pediatric-onset AI experience AC. Younger age and an increased number of infections are independent risk factors for developing AC in these patients.
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