title: Adrenal function in thalassemia major adolescents.
abstract: 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.
  
  
  
  We measured adrenal androgen response along with cortisol to the standard (250 mg) dose ACTH test.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Paediatrics Department, Ain Shams University, Cairo, Egypt. hebased@yahoo.com
authors.forename: Heba H
authors.initials: HH
authors.lastname: Elsedfy
authors.forename: M
authors.initials: M
authors.lastname: El Kholy
authors.forename: R T
authors.initials: RT
authors.lastname: Hamza
authors.forename: Al
authors.initials: A
authors.lastname: Hamed
authors.forename: M
authors.initials: M
authors.lastname: Elalfy
mesh_terms: D000293:Adolescent; D000311:Adrenal Glands / Q000378:metabolism / Q000503:physiopathology*; D000309:Adrenal Insufficiency / Q000097:blood / Q000209:etiology* / Q000503:physiopathology*; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000735:Androstenedione / Q000097:blood; D002648:Child; D003687:Dehydroepiandrosterone / Q000097:blood; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D008297:Male; D015995:Prevalence; D055815:Young Adult; D017086:beta-Thalassemia / Q000150:complications* / Q000503:physiopathology*
chemical_list: D000735:Androstenedione; D003687:Dehydroepiandrosterone; D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone