title: The Frequency of Adrenal Insufficiency in Adolescents and Young Adults with Thalassemia Major versus Thalassemia Intermedia in Iran.
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 primary 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 median and interquartile range of serum ferritin levels were 2184±3700 ng/ml and 437±443ng/ml 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 in cortisol secretion at the basal state. However, cortisol response to standard - dose ACTH was normal in all patients with TM and TI.
authors.affiliation: Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Sara
authors.initials: S
authors.lastname: Matin
authors.affiliation: Department of Anesthesiology, Jahrom University of Medical Science, Jahrom, Iran.
authors.forename: Masoud Ghanei
authors.initials: MG
authors.lastname: Jahromi
authors.affiliation: Metabolic and Endocrinology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Zohreh
authors.initials: Z
authors.lastname: Karemizadeh
authors.affiliation: Hematology Research Center, Shiraz University of Medical Science, Shiraz, Iran.
authors.forename: Sezaneh
authors.initials: S
authors.lastname: Haghpanah
authors.affiliation: Pediatric and Adolescent Outpatient Clinic, Quisisana Private Accredited Hospital, Ferrara (Italy).
authors.forename: Vincenzo
authors.initials: V
authors.lastname: De Sanctis
authors.affiliation: Department of Pediatrics, Alexandria University Children's Hospital, Alexandria, Egypt.
authors.forename: Ashraf
authors.initials: A
authors.lastname: Soliman
authors.affiliation: Hematology Research Center, Shiraz University of Medical Science, Shiraz, Iran.
authors.forename: Javad
authors.initials: J
authors.lastname: Dehbozorgian
authors.affiliation: Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, Iran.
authors.forename: Zahra
authors.initials: Z
authors.lastname: Majd
authors.affiliation: Hematology Research Center, Shiraz University of Medical Science, Shiraz, Iran.
authors.forename: Narges
authors.initials: N
authors.lastname: Rezaei
authors.affiliation: Hematology Research Center, Shiraz University of Medical Science, Shiraz, Iran.
authors.forename: Mehran
authors.initials: M
authors.lastname: Karimi