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Leukocyte Telomere Length in Children With Congenital Adrenal Hyperplasia
Christina Raftopoulou1,2, Ozair Abawi3, Grit Sommer4
1Division of Endocrinology, Metabolism and Diabetes, First Department of Pediatrics, National and Kapodistrian University of Athens Medical School, 'Aghia Sophia' Children's Hospital, Athens 11527, Greece.
Insights
Leukocyte telomere length (LTL) is shorter in children with classic congenital adrenal hyperplasia (CAH) and those overtreated with glucocorticoids. This finding suggests LTL may serve as a biomarker for monitoring CAH treatment.
Area of Science:
- Pediatric Endocrinology
- Genetics
- Molecular Biology
Background:
- Exposure to chronic stress and hypercortisolism is linked to shorter leukocyte telomere length (LTL), a marker of biological aging and cardiovascular disease risk.
- Children with congenital adrenal hyperplasia (CAH) require glucocorticoid treatment, which can lead to varying levels of exposure.
Purpose of the Study:
- To investigate the association between congenital adrenal hyperplasia (CAH) and leukocyte telomere length (LTL) in children.
- To determine if glucocorticoid treatment status (undertreated, optimally treated, overtreated) influences LTL in pediatric CAH patients.
Main Methods:
- A prospective observational cohort study involving 76 children with genetically confirmed CAH across four academic pediatric endocrinology clinics.
- Leukocyte telomere length (LTL) was measured using quantitative real-time PCR at two follow-up visits.
- Clinical and endocrinologic evaluations were performed to classify treatment status, with data analyzed using linear mixed models adjusted for age, sex, and BMI-z.
Main Results:
- Patients with classic CAH had significantly shorter LTL compared to those with nonclassic CAH.
- Overtreatment with glucocorticoids was associated with shorter LTL compared to optimal treatment.
- Treatment with prednisolone resulted in shorter LTL than treatment with hydrocortisone.
Conclusions:
- Leukocyte telomere length (LTL) is reduced in children with classic CAH and those receiving excessive glucocorticoid therapy.
- These findings highlight the potential of LTL as a biomarker for assessing the adequacy of glucocorticoid treatment in CAH.
- Monitoring LTL may aid in optimizing treatment strategies to mitigate long-term health risks associated with glucocorticoid exposure.
Context:
Exposure to chronic stress and hypercortisolism is associated with decreased leukocyte telomere length (LTL), a marker for biological aging and cardiovascular disease. Children with congenital adrenal hyperplasia (CAH) are treated with glucocorticoids.
Objective:
To investigate LTL in children with CAH.
Methods:
In this prospective observational cohort study, conducted at 4 academic pediatric endocrinology outpatient clinics, children with genetically confirmed CAH were assessed at 2 follow-up visits (mean 4.1 ± 0.7 months apart). At each visit, LTL was determined by quantitative real-time PCR. All subjects underwent detailed clinical and endocrinologic evaluation and were classified as undertreated, optimally treated, or overtreated, accordingly. The influence of clinical factors on LTL was investigated using linear mixed models adjusted for age, sex, and BMI-z.
Results:
We studied 76 patients, of whom 31 (41%) were girls, 63 (83%) had classic CAH, 67 (88%) received hydrocortisone, and 8 (11%) prednisolone. Median age at first visit was 12.0 years (IQR, 6.3-15.1), and median BMI-z was 0.51 (IQR, -0.12 to 1.43). LTL was shorter in patients with classic vs nonclassic CAH (-0.29, P = 0.012), in overtreated than in optimally treated patients (-0.07, P = 0.002), and patients receiving prednisolone compared with hydrocortisone (-0.34, P < 0.001). LTL was not associated with undertreatment or daily hydrocortisone-equivalent dose (P > 0.05).
Conclusion:
LTL is shorter in patients with classic than nonclassic CAH, and in those who are overtreated with hydrocortisone or treated with long-acting glucocorticoids. These findings may be attributed to chronic exposure to supraphysiologic glucocorticoid concentrations and indicate that LTL may be used as a biomarker for monitoring glucocorticoid treatment.
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