地中海贫血重症患者青春期状态及其与血清铁蛋白水平的关系

IF 0.4 Q4 PEDIATRICS
M. A. Aghamaleki, A. Tamaddoni, H. M. Nesheli, M. Hajiahmadi, Motahareh Amouzadeh Samakoush, Faeze Aghajanpour
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引用次数: 2

摘要

背景:地中海贫血(Thalassemia major, TM)是一种最常见的遗传性贫血,伴有多种内分泌疾病(尤其是性腺功能减退)。因此,我们评估了青春期延迟发生率及其与血清铁蛋白水平的关系。材料与方法:本横断面(描述性分析)研究对2016年在伊朗巴博勒Amirkola地中海贫血中心收治的100例14-64岁的TM患者进行了研究。评估青少年的青春期状态、Marshall-Tanner量表、DP的存在程度及其不同类型。测定血清平均铁蛋白水平,按2500 ng/ml分为3组。数据分析采用SPSS (version20)软件。结果:100例患者中,女性64例(64%),男性36例(36%)。年龄方面,20岁以下和20岁以上分别有23、77例(%)。DP 69例(69%),其中继发性(中枢性)促性腺功能减退64例(92.8%)。平均血清铁蛋白水平(±SD)为2707.94±1683.42 ng/ml。此外,26例、29例和45例患者铁蛋白水平分别为2500 ng/ml。32例(46.4%)DP患者铁蛋白水平高于2500 ng/ml (p值= 0.623)。结论:TM患者DP发生率高,需要在青春期进行仔细检查和随访,早期诊断,及时治疗,提高生活质量,预防骨质疏松等并发症的发生。我们没有发现血清铁蛋白水平与性腺功能减退有任何显著的关系,即使在服用足够的铁螯合剂的病例中也是如此。关键词:青春期延迟,铁蛋白,促性腺功能亢进症,促性腺功能亢进症,地中海贫血
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Pubertal status and its relation with serum ferritin level in thalassemia major patients
Background: Thalassemia major (TM) is one of the most common hereditary anemia with multiple endocrinopathies (especially hypogonadism). So, we evaluated the rate of delayed puberty (DP) and its relation with serum ferritin level in patients. Materials and Methods: This cross-sectional (descriptive-analytical) study was conducted on 100 patients with TM between 14-64 years old, admitted to Amirkola Thalassemia Center, Babol, Iran, in 2016. The pubertal status, (Marshall-Tanner scale), existance of DP, and its different types were evaluated. Mean serum ferritin level was measured and the data were classified to three groups of <1500, 1500-2500, and >2500 ng/ml. Data were analyzed using SPSS (version20). Results: Out of 100 patients, 64 (64%) and 36 (36%) were female and male, respectively. Considering age, 23, 77 patients (%) were under and over 20 years old, respectively. Totally, 69 (69%) of them had DP, of whom 64 (92.8%) ones had secondary (central) hypogonadotropic hypogonadism. Mean serum ferritin level (±SD) was 2707.94±1683.42 ng/ml. In addition, 26, 29, and 45 patients had ferritin level <1500, 1500-2500, and >2500 ng/ml, respectively. Thirty two patients with DP (46.4%) had ferritin level above 2500 ng/ml (p-value= 0.623). Conclusion: The results showed a high frequency of DP in TM patients, requiring careful examination and follow-up in terms of puberty for early diagnosis and proper treatment to improve their quality of life, and prevention of the complications like osteoporosis. We couldn't find any significant relationship between serum ferritin level and hypogonadism, even for cases who received enough iron chelators. Keywords: Delayed Puberty, Ferritin, Hypergonadotropic Hypogonadism, Hypogonadotropic Hypogonadism, Thalassemia Major    
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来源期刊
CiteScore
0.80
自引率
33.30%
发文量
33
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