title: Assessment of adrenal endocrine function in Asian thalassemics.
abstract: Adrenal endocrine function was assessed in a cohort of 20 patients, between 10 and 20 years of age, with transfusion dependent beta thalassemia. Cortisol levels were assayed before and after ACTH stimulation with 1 micrograms and 250 micrograms. Adrenal dysfunction was defined as a basal cortisol of greater than 400 nmol/L and/or peak cortisol levels of greater than 500 nmol/L. Overall, 9 patients (45 %) had in vitro evidence of adrenal dysfunction. A statistical significant correlation (r=0.4308; P < 0.05), between wasting and the basal cortisol level, was observed. Similarly, there was correlation between the number of transfusions received and growth failure (r=0.4774;P < 0.05). In comparison to the involvement of other endocrine axes in polytransfused thalassemics, the adrenal endocrine function abnormalities are minor and clinically of little consequence. The observations, albeit, in a small cohort of thalassemics, stress the need for an annual estimation of basal cortisol level, especially in patients with wasting.
authors.affiliation: Department of Pediatrics (Hematology-Oncology Unit), Post Graduate Institute of Medical Education and Research, Chandigarh 160 012, India.
authors.forename: Arun
authors.initials: A
authors.lastname: Srivatsa
authors.forename: R K
authors.initials: RK
authors.lastname: Marwaha
authors.forename: R
authors.initials: R
authors.lastname: Muraldharan
authors.forename: Amita
authors.initials: A
authors.lastname: Trehan
mesh_terms: D000293:Adolescent; D000309:Adrenal Insufficiency / Q000378:metabolism / Q000503:physiopathology*; D000328:Adult; D044466:Asian People; D002648:Child; D004703:Endocrine System / Q000378:metabolism / Q000503:physiopathology*; D005260:Female; D006801:Humans; D006854:Hydrocortisone; D007501:Iron / Q000009:adverse effects*; D008297:Male; D018570:Risk Assessment; D017086:beta-Thalassemia / Q000378:metabolism / Q000503:physiopathology*
chemical_list: D007501:Iron; D006854:Hydrocortisone