THE FREQUENCY OF ADRENAL INSUFFICIENCY IN ADOLESCENTS AND YOUNG ADULTS WITH THALASSEMIA MAJOR VERSUS THALASSEMIA INTERMEDIA IN IRAN

Sara Matin, Masoud Ghanei Jahromi, Zohreh Karemizadeh, Sezaneh Haghpanah, Vincenzo De Sanctis, Ashraf Soliman, Mehran Karimi
  • Sara Matin
    Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, Iran, Iran, Islamic Republic of
  • Masoud Ghanei Jahromi
    Department of Anesthesiology, Jahrom University of Medical Science, Jahrom, Iran, Iran, Islamic Republic of
  • Zohreh Karemizadeh
    Affiliation not present
  • Sezaneh Haghpanah
    Hematology Research Center, Shiraz University of Medical Science, Shiraz, Iran., Iran, Islamic Republic of
  • Vincenzo De Sanctis
    Pediatric and Adolescent Outpatient Clinic, Quisisana Private Accredited Hospital, Ferrara (Italy), Iran, Islamic Republic of
  • Ashraf Soliman
    Department of Pediatrics, Alexandria University Children’s Hospital, Alexandria, Egypt., Iran, Islamic Republic of
  • Mehran Karimi
    Hematology Research Center, Shiraz University of Medical Science, Shiraz, Iran., Iran, Islamic Republic of | karimim@sums.ac.ir

Abstract

Background: Endocrine dysfunction is not uncommon complication in patients with transfusion dependent thalassemia and is thought to occur as a consequence of excessive iron overload. The main objective of this study is to determine the frequency of adrenal insufficiency in patients with thalassemia major and thalassemia intermediate. Methods: This cross-sectional study was done at the Shiraz University of Medical Sciences, Shiraz, Southern Iran, in 2013. One hundred and ninety patients were divided into two groups; thalassemia major(TM) and thalassemia intermediate (TI) groups. We measured 8 AM serum cortisol, ACTH and ferritin concentrations in all patients. Results: The mean age of the TM and TI group were 22.5±5.7 and 23.8±6 years, respectively. 90 patients (47.4%) were splenectomized,  34 (36.2%) with TM and  56 (58.2%) with TI (p : 0.001). The mean serum ferritin levels were 3056.5±2306 and 666.2±616.5 in TM and TI respectively (p: 0.001).  Three patients with TM (1.6%) had low basal cortisol and ACTH levels. However their cortisol response to ACTH stimulation was normal. Conclusions: Low basal concentrations of cortisol and ACTH occurred in 1.6% of our adolescents young adult patients with TM suggesting a central defect of cortisol secretion at the basal state. However, cortisol response to standard – dose ACTH was normal in all patients with TM and TI.

Keywords

adrenal insufficiency; thalassemia intermediate (TI); thalassemia major (TM)

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Submitted: 2014-08-29 14:23:51
Published: 2014-08-28 00:00:00
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