Hagag Adel A, Badraia Ibrahim M, Elfarargy Mohamed S, Abo El-Enein Amany M
16 Elemam Moslem street branched from Elhelw Street, Tanta, Gharbia, Egypt.
Endocr Metab Immune Disord Drug Targets. 2016;16(2):148-153. doi: 10.2174/1871530316666160506150516.
Beta-thalassemia is 'a hereditary blood disorder characterized by reduced or absent beta globin chain synthesis, resulting in reduced hemoglobin in red blood cells, decreased RBCs production and anemia'. Patients with thalassemia major require repeated blood transfusions which 'lead to accumulation of iron in different tissues, including tissues of endocrine glands'. This study aims to evaluate serum gonadal hormones levels in adolescent females with β-thalassemia in relation to iron overload.
This study was conducted on 80 adolescent females with β-thalassemia having serum ferritin over 1000 ng/ml with range of their ages between 11 -15 years and mean age of 12. 42 ± 1.12 years (Group I) and 80 females with β-thalassemia of matched age having serum ferritin less than 500 ng/ml (Group II). For all patients the following were done: Complete blood count, hemoglobin electrophoresis, serum iron status including 'serum ferritin, serum iron and total iron binding capacity and' gonadal hormones including LH, FSH, and serum Estrogen.
'There were significantly higher serum ferritin and serum iron and significantly lower TIBC', Follicular Stimulating Hormone, Luteinizing Hormone and Estrogen levels in Group I compared with Group II (Mean serum ferritin was 1839.5 ± 258.2 ng/ml in group I versus 336.2 ± 33.5 ng/ml in group II with p value of 0.001, mean serum iron was 201.3 ± 38.43 ug/dl in group I versus 124.47 ± 12.23 ug/dl in group II with p value of 0.001, mean serum total iron binding capacity was 252.56 ± 23.21 ug/dl in group I versus 353.6 ± 31.79 ug/dl in group II with p value of 0.001, mean FSH level was 1.17 ± 0.67 mIU/ml in group I versus 2.55 ± 1.92 mIU/ml group II with p value of 0.029, mean LH level was 0.98 ± 0.25 mIU/ml in group I versus 1.91 ± 0. 42 mIU /ml in group II with p value of 0.016, mean Estrogen level was 22.46 ± 6.36 pg/ml in group I versus 35 .63 ± 8.63 pg/ml in group II with p value of 0.010). There were significant negative correlations between gonadal hormones including serum Follicle-Stimulating Hormone, Luteinizing Hormone, Estrogen and serum ferritin (r = - 0. 835 and p value of 0.01 for FSH and serum ferritin, r = -0. 949 and p value of 0.01 for LH and serum ferritin and r= - 0. 900 and p value of p 0.01 for Estrogen and serum ferritin.
Female patients with β-thalassemia with iron overload may have gonadal hormones deficiency with significant negative correlation between gonadal hormones and serum ferritin.
Regular iron chelation to prevent iron overload with subsequent irreversible damage of the ovaries and also regular follow up for females with β-thalassemia with assessment of puberty as they are more vulnerable to develop hypogonadism and may require hormonal replacement therapy.
β地中海贫血是一种遗传性血液疾病，其特征是β珠蛋白链合成减少或缺乏，导致红细胞中的血红蛋白减少、红细胞生成减少和贫血。重型地中海贫血患者需要反复输血，这会导致铁在不同组织中蓄积，包括内分泌腺组织。本研究旨在评估铁过载情况下，青少年女性β地中海贫血患者的血清性腺激素水平。
本研究纳入了80名血清铁蛋白超过1000 ng/ml的青少年女性β地中海贫血患者，年龄在11 - 15岁之间，平均年龄为12.42±1.12岁（第一组），以及80名年龄匹配、血清铁蛋白低于500 ng/ml的β地中海贫血女性（第二组）。对所有患者进行了以下检查：全血细胞计数、血红蛋白电泳、血清铁状态（包括血清铁蛋白、血清铁和总铁结合力）以及性腺激素（包括促黄体生成素、促卵泡生成素和血清雌激素）。
与第二组相比，第一组患者的血清铁蛋白和血清铁显著升高，总铁结合力、促卵泡生成素、促黄体生成素和雌激素水平显著降低（第一组血清铁蛋白平均值为1839.5±258.2 ng/ml，第二组为336.2±33.5 ng/ml，p值为0.001；第一组血清铁平均值为201.3±38.43 ug/dl，第二组为124.47±12.23 ug/dl，p值为0.001；第一组血清总铁结合力平均值为252.56±23.21 ug/dl，第二组为353.6±31.79 ug/dl，p值为0.001；第一组促卵泡生成素平均水平为1.17±0.67 mIU/ml，第二组为2.55±1.92 mIU/ml，p值为0.029；第一组促黄体生成素平均水平为0.98±0.25 mIU/ml，第二组为1.91±0.42 mIU/ml，p值为0.016；第一组雌激素平均水平为22.46±6.36 pg/ml，第二组为35.63±8.63 pg/ml，p值为0.010）。性腺激素（包括血清促卵泡生成素、促黄体生成素、雌激素）与血清铁蛋白之间存在显著负相关（促卵泡生成素与血清铁蛋白的r = - 0.835，p值为0.01；促黄体生成素与血清铁蛋白的r = -0.949，p值为0.01；雌激素与血清铁蛋白的r = - 0.900，p值为0.01）。
铁过载的女性β地中海贫血患者可能存在性腺激素缺乏，性腺激素与血清铁蛋白之间存在显著负相关。
定期进行铁螯合治疗以防止铁过载及随后卵巢的不可逆损伤，同时对女性β地中海贫血患者进行定期随访，评估青春期发育情况，因为她们更容易发生性腺功能减退，可能需要激素替代治疗。
