Abstract : Patients with β-thalassemia major (β-TM) often present with endocrine abnormalities, mainly due to dysfunction in their hypothalamic-pituitary axis, such as delayed growth and puberty. We aimed to assess the growth parameters of patients with β-TM and to evaluate the growth hormone (GH)- insulin like growth factor-1 (IGF-1) axis. This case-control study included 50 patients, 8-19 years old, with β-TM registered at Basra Center for Hereditary Blood Diseases, Southern Iraq, and 75 apparently healthy subjects. Anthropometric data were evaluated using the WHO Child Growth Standards. Growth hormone provocation test, serum IGF-1, ferritin, thyroid and gonadotropin hormones were also measured. Twenty-six (52%) β-TM patients had short stature. Patients with β-TM had significantly lower peak GH levels (after induction) and IGF-1 levels compared to the control group, (P < 0.01 and P < 0.001, respectively). Growth hormone deficiency and impaired IGF-1 were found in 65% and 92% of patients with short stature, respectively. The GH deficiency was observed after a provocation test with a cut- off peak less than 7 or 10 ng/ml. Moreover, significant negative associations were reported between serum ferritin and peak GH (r - 0.239), IGF-1 (r - 0.386), thyroxine (r - 0.423), and hemoglobin (r - 0.612) levels. IGF-1 can be considered as a useful and sensitive test in assessing growth retardation among pediatric patients with β-TM. In addition, more than one-third of patients were GH-sufficient, suggesting a multifactorial origin rather than GH deficiency alone.
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