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Adrenal function in thalassemia major adolescents
Heba H Elsedfy1, M El Kholy, R T Hamza
1Paediatrics Department, Ain Shams University, Cairo, Egypt. hebased@yahoo.com
Pediatric Endocrinology Reviews : PER
|June 28, 2011
Summary
Adrenal insufficiency affects 15.5% of beta-thalassemia patients. Adrenal androgen production decreases with puberty, potentially explaining poor hair development in adolescents with thalassemia.
Area of Science:
- Endocrinology
- Hematology
- Pediatrics
Background:
- Thalassemia is associated with a high prevalence of adrenal insufficiency (18-45%).
- Previous research indicated a dissociation between cortisol and adrenal androgen secretion in beta-thalassemia patients.
Purpose of the Study:
- To evaluate adrenal androgen response alongside cortisol levels in beta-thalassemia patients using a standard ACTH stimulation test.
- To investigate the relationship between adrenal function and pubertal development in adolescents with beta-thalassemia.
Main Methods:
- Forty-five beta-thalassemia major patients (ages 12-20) underwent a 250 mg cosyntropin (ACTH) test.
- Blood samples were collected pre- and post-stimulation to measure total cortisol, dehydroepiandrosterone (DHEA), and androstenedione (A).
- Adrenal insufficiency was defined based on standard criteria, and androgen levels were correlated with Tanner stage.
Main Results:
- Adrenal insufficiency was identified in 7 of 45 (15.5%) patients.
- Adrenal androgen levels (DHEA and A) significantly decreased with advancing Tanner stage.
- No significant differences in anthropometric or laboratory parameters were observed between patients with and without adrenal insufficiency.
Conclusions:
- Adrenal insufficiency is a notable complication in thalassemia patients.
- Declining adrenal androgen production during puberty in thalassemic adolescents may contribute to the observed poor development of secondary sexual hair.
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