What is it about?
Homozygous beta-thalassemia major (hbeta-TM) is an autosomal recessive hemoglobinopathy with prominent endocrine complications. Our aims were to evaluate reproductive hormones, hypothalamic-pituitary-ovarian axis, and fertility in female patients with hbeta-TM.
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Why is it important?
Modern therapeutic approaches of b-TM through frequent blood transfusions and regular iron chelation have significantly increased the average lifespan and improved the quality of life of hb-TM patients. Multiple endocrine abnormalities develop during the course of hb-TM, which are attributed mainly to iron overload. Other proposed mechanisms for hypothalamic-pituitary gonadal (HPG) axis altered functionality are physical and psychologic stress, coexisting infections, involvement of liver and spleen, nutritional problems, abnormal conversion of steroid hormones to their active metabolites, and defective hepatic biosynthesis of insulin-like growth factor-1 (IGF-1). In the pituitary gland, iron toxicity mainly affects the gonadotropin-secreting cells. The condition has been suggested to result from selective iron deposition in the pituitary gonadotrophs and hypothalamus.
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This page is a summary of: Reproductive Hormones and Hypothalamic-Pituitary-Ovarian Axis in Female Patients With Homozygous β-Thalassemia Major, Journal of Pediatric Hematology/Oncology, May 2010, Wolters Kluwer Health, DOI: 10.1097/mph.0b013e3181cf8156.
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