BACKGROUND: Several studies reported a significant prevalence of adrenal insufficiency, ranging from 18-45%, in patients with thalassemia. Evidence for dissociation of cortisol and adrenal androgen secretion in patients with beta-thalassemia was previously reported. AIM: We measured adrenal androgen response along with cortisol to the standard (250 mg) dose ACTH test. METHODS: Forty five beta-thalassemia major (TM) patients were enrolled. Their ages ranged between 12 and 20 years (14.9 ± 2.2 years). All patients underwent the 250 mg cosyntropin test in the morning before blood transfusion. Blood samples for total cortisol, dehdroepiandrosterone (DHEA) and androstendione (A) measurements were collected before and 60 min after IV injection of 250 mg cosyntropin. Adrenal insufficiency was observed in 7 of 45 (15.5%) patients. Adrenal androgen levels decreased significantly with advancing Tanner stage. No difference was noted between patients with and without adrenal insufficiency regarding anthropometric and laboratory parameters. CONCLUSION: Adrenal insufficiency is not a rare complication in thalassemia. Adrenal androgen production declines with advancing puberty in thalassemic adolescents and might explain the poor development of pubic and axillary hair observed in this condition.
BACKGROUND: Several studies reported a significant prevalence of adrenal insufficiency, ranging from 18-45%, in patients with thalassemia. Evidence for dissociation of cortisol and adrenal androgen secretion in patients with beta-thalassemia was previously reported. AIM: We measured adrenal androgen response along with cortisol to the standard (250 mg) dose ACTH test. METHODS: Forty five beta-thalassemia major (TM) patients were enrolled. Their ages ranged between 12 and 20 years (14.9 ± 2.2 years). All patients underwent the 250 mg cosyntropin test in the morning before blood transfusion. Blood samples for total cortisol, dehdroepiandrosterone (DHEA) and androstendione (A) measurements were collected before and 60 min after IV injection of 250 mg cosyntropin. Adrenal insufficiency was observed in 7 of 45 (15.5%) patients. Adrenal androgen levels decreased significantly with advancing Tanner stage. No difference was noted between patients with and without adrenal insufficiency regarding anthropometric and laboratory parameters. CONCLUSION:Adrenal insufficiency is not a rare complication in thalassemia. Adrenal androgen production declines with advancing puberty in thalassemic adolescents and might explain the poor development of pubic and axillary hair observed in this condition.
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Authors: Vincenzo De Sanctis; Ashraf T Soliman; Heba Elsedfy; Alice Albu; Soad Al Jaouni; Saif Al Yaarubi; Salvatore Anastasi; Duran Canatan; Massimo Di Maio; Salvatore Di Maio; Mohamed El Kholy; Mehran Karimi; Doaa Khater; Yurdanur Kilinc; Su Han Lum; Nicos Skordis; Praveen Sobti; Iva Stoeva; Ploutarchos Tzoulis; Yasser Wali; Christos Kattamis Journal: Mediterr J Hematol Infect Dis Date: 2016-07-01 Impact factor: 2.576
Authors: Vincenzo De Sanctis; Ashraf T Soliman; Heba Elsedfy; Nicos Skordis; Christos Kattamis; Michael Angastiniotis; Mehran Karimi; Mohd Abdel Daem Mohd Yassin; Ahmed El Awwa; Iva Stoeva; Giuseppe Raiola; Maria Concetta Galati; Elsaid M Bedair; Bernadette Fiscina; Mohamed El Kholy Journal: Indian J Endocrinol Metab Date: 2013-01