Al-Rimawi H S, Jallad M F, Amarin Z O, Obeidat B R
Department of Pediatrics, Faculty of Medicine, Jordan University of Science and Technology, Irbid, Jordan.
Int J Gynaecol Obstet. 2005 Jul;90(1):44-7. doi: 10.1016/j.ijgo.2005.03.024.
To evaluate the function of the hypothalamic-pituitary-gonadal axis in adolescent female patients with beta-thalassemia major.
A prospective study of the function of the hypothalamic-pituitary-gonadal axis function of 31 beta-thalassemia major females aged between 13 and 22 years and in 12 control females aged between 12 and 22 years. All were treated at Princess Rahma Teaching Hospital, Irbid, Northern Jordan between April 2001 and April 2003.
Of the 31 beta-thalassemia major females, 13 (41.9%) had delayed puberty. Hypothalamic-pituitary-ovarian axis dysfunction was found in 15 patients (48.4%). Twelve patients (38.7%) had hypogonadotropic hypogonadism and 5 (16.1%) had ovarian failure. High levels of serum ferritin were significantly higher in patients with delayed puberty.
Pituitary and ovarian dysfunction are common problems in beta-thalassemia major patients. The main possible cause is iron overload. This stresses the need for intensive and regular use of chelation therapy to prevent damage to the hypothalamic-pituitary-ovarian axis.
评估重型β地中海贫血青少年女性患者下丘脑-垂体-性腺轴的功能。
对31例年龄在13至22岁之间的重型β地中海贫血女性患者以及12例年龄在12至22岁之间的对照女性患者的下丘脑-垂体-性腺轴功能进行前瞻性研究。所有患者均于2001年4月至2003年4月在约旦北部伊尔比德的拉赫玛公主教学医院接受治疗。
31例重型β地中海贫血女性患者中，13例（41.9%）青春期延迟。15例患者（48.4%）存在下丘脑-垂体-卵巢轴功能障碍。12例患者（38.7%）为低促性腺激素性性腺功能减退，5例（16.1%）为卵巢功能衰竭。青春期延迟患者的血清铁蛋白水平显著更高。
垂体和卵巢功能障碍是重型β地中海贫血患者的常见问题。主要可能原因是铁过载。这强调了需要强化并定期使用螯合疗法以预防下丘脑-垂体-卵巢轴受损。
