CONTEXT: High prevalence of "biochemical" adrenal insufficiency (AI) in thalassemics has been reported. However, "clinical" AI is rare. AIM: The aim was to determine whether cortisol binding globulin (CBG) or tests used in assessing adrenal function contributed to the abnormally high prevalence of biochemical AI. SETTING: The study was conducted at Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. PARTICIPANTS: Participants included 56 children and adolescents with thalassemia and 44 controls. MAIN OUTCOME MEASURES: Serum CBG and adrenal function test results assessed by 1 μg cosyntropin test and insulin tolerance test (ITT) were measured. Free cortisol index (FCI) calculated by total cortisol (TC)/CBG and calculated free cortisol (cFC) were determined. RESULTS: Mean (sd) CBG levels were comparable between patients and controls [45.2 (11.0) vs. 47.0 (8.6) mg/liter]. Peak TC, FCI, and cFC after cosyntropin test were lower in thalassemics [TC, 15.2 (4.0) vs. 18.9 (3.1) μg/dl; FCI, 3.4 (0.8) vs. 4.2 (1.2) μg/mg, P <0.001; and cFC, 1.03 (0.38) vs. 1.44 (0.61) μg/dl, P = 0.008]. Thirty of 56 thalassemics (53.6%) had AI, defined as having peak TC of less than 16 μg/dl. ITT was performed in 26 of those 30 patients. Five of 26 patients had peak TC after an ITT of at least 20 μg/dl. As a result, the estimated frequency of AI in the entire patient group was reduced by approximately 10%. CONCLUSION: The 1 μg cosyntropin test could be an adrenal function screening test in thalassemics. However, for definite diagnosis, ITT should be performed in those having peak total cortisol of less than 16 μg/dl after the 1 μg cosyntropin test.
CONTEXT: High prevalence of "biochemical" adrenal insufficiency (AI) in thalassemics has been reported. However, "clinical" AI is rare. AIM: The aim was to determine whether cortisol binding globulin (CBG) or tests used in assessing adrenal function contributed to the abnormally high prevalence of biochemical AI. SETTING: The study was conducted at Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. PARTICIPANTS: Participants included 56 children and adolescents with thalassemia and 44 controls. MAIN OUTCOME MEASURES: Serum CBG and adrenal function test results assessed by 1 μg cosyntropin test and insulin tolerance test (ITT) were measured. Free cortisol index (FCI) calculated by total cortisol (TC)/CBG and calculated free cortisol (cFC) were determined. RESULTS: Mean (sd) CBG levels were comparable between patients and controls [45.2 (11.0) vs. 47.0 (8.6) mg/liter]. Peak TC, FCI, and cFC after cosyntropin test were lower in thalassemics [TC, 15.2 (4.0) vs. 18.9 (3.1) μg/dl; FCI, 3.4 (0.8) vs. 4.2 (1.2) μg/mg, P <0.001; and cFC, 1.03 (0.38) vs. 1.44 (0.61) μg/dl, P = 0.008]. Thirty of 56 thalassemics (53.6%) had AI, defined as having peak TC of less than 16 μg/dl. ITT was performed in 26 of those 30 patients. Five of 26 patients had peak TC after an ITT of at least 20 μg/dl. As a result, the estimated frequency of AI in the entire patient group was reduced by approximately 10%. CONCLUSION: The 1 μg cosyntropin test could be an adrenal function screening test in thalassemics. However, for definite diagnosis, ITT should be performed in those having peak total cortisol of less than 16 μg/dl after the 1 μg cosyntropin test.
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Authors: Ashraf T Soliman; Mohamed Yassin; Nadra M S Abdel Majuid; Aml Sabt; Mohamed O Abdulrahman; Vincenzo De Sanctis Journal: Indian J Endocrinol Metab Date: 2013-11
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