Karamifar Hamdollah, Shahriari Mehdi, Amirhakimi Gholam Hossein
Turk J Haematol. 2005 Jun 5;22(2):65-9.
Thalassemia major is a severe progressive hemolytic anemia and a serious medical problem worldwide. Endocrine dysfunctions are well described in patients with thalassemia major. Data for endocrine complications from developing countries are scant. Endocrine complications in developing countries may be frequent due to suboptimal iron chelation. The aim of this study was to evaluate the prevalence of delayed puberty and growth failure in patients with beta-thalassemia major. We evaluated the growth and sexual development of 146 patients with thalassemia major aged 10-22 years. The following data were recorded in questionnaire, age, sex, height, weight, serum ferritin levels and pubertal staging. Failure of puberty was present in 75.6% of boys and 68.4% of girls aged 12-22 years. Gonadotropin insufficiency was found in most of the patients with lack of puberty. There was a significant difference between the height of patients with pubertal development (153 ± 9.1 cm) and those with delayed puberty (140 ± 9.1), (p< 0.001). Short stature was present in 65.7% of patients. Sixty-nine percent of boys and 62.9% of girls were found to be less than 2 SD below the mean for normal height; after the age of 12, the percentage was 78.9% in girls and 83.8% in boys after the age of 14. We conclude that failure of puberty and impaired growth are very common in our thalassemic patients which necessitates newer protocols of treatment, correct blood transfusion and chelation therapy.
重型地中海贫血是一种严重的进行性溶血性贫血,是全球范围内一个严重的医学问题。重型地中海贫血患者的内分泌功能障碍已有详尽描述。来自发展中国家的内分泌并发症数据匮乏。由于铁螯合治疗欠佳,发展中国家的内分泌并发症可能很常见。本研究的目的是评估重型β地中海贫血患者青春期发育延迟和生长发育迟缓的患病率。我们评估了146例年龄在10至22岁之间的重型地中海贫血患者的生长和性发育情况。通过问卷记录了以下数据:年龄、性别、身高、体重、血清铁蛋白水平和青春期分期。12至22岁的男孩中75.6%以及女孩中68.4%存在青春期发育失败。大多数青春期发育缺失的患者存在促性腺激素不足。青春期发育患者的身高(153±9.1厘米)与青春期发育延迟患者的身高(140±9.1厘米)之间存在显著差异(p<0.001)。65.7%的患者身材矮小。69%的男孩和62.9%的女孩身高低于正常均值2个标准差;12岁以后,女孩的这一比例为78.9%,14岁以后男孩的这一比例为83.8%。我们得出结论,青春期发育失败和生长发育受损在我们的地中海贫血患者中非常常见,这需要更新治疗方案、正确的输血和螯合治疗。