What is it about?

The endocrine profile, hypothalamus-pituitary-testis axis and semen quality were evaluated in male patients with homozygous -thalassemia major.

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Why is it important?

Homozygous beta-TM is an autosomal recessive hemoglobinopathy with the highest prevalence in the Mediterranean countries, India and Southeast Asia. The cause is a genetic mutation that decreases or even halts the synthesis of beta-globin chains. Clinical management for hbeta-TM changed radically when a regular transfusion program was introduced in 1964 by Wolman. The combination of transfusion and chelation therapy has dramatically extended the life expectancy of patients with thalassemia. However, repeat transfusions lead to iron accumulation in the liver, pituitary gland and other organs6 in the form of ferritin or hemosiderin. The most prominent endocrine complications are growth retardation and the failure of normal pubertal development. A lack of sexual maturation and loss of gonadal function occur frequently in these patients. In the pituitary gland iron toxicity mainly affects the gonadotropin secreting cells and, therefore, in patients with hbetaTM HH is a common abnormality.


HH, infertility and growth retardation are common problems in patients with hbeta-TM. At least investigation of the HPT axis, semen analysis and close annual followup seem warranted in these patients. Further studies are needed to clarify the causes of the results in this study and find the main mechanisms responsible for HH in thalassemia cases.

Dr Mohammad Reza Safarinejad
University of Medical Sceices

Read the Original

This page is a summary of: Evaluation of Semen Quality, Endocrine Profile and Hypothalamus-Pituitary-Testis Axis in Male Patients With Homozygous β-Thalassemia Major, The Journal of Urology, June 2008, Wolters Kluwer Health,
DOI: 10.1016/j.juro.2008.01.103.
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