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孟加拉国输血依赖型β地中海贫血患者性腺功能减退的患病率:探讨血清铁蛋白水平作为诊断工具的作用。

Prevalence of hypogonadism in transfusion-dependent β-thalassemia patients of Bangladesh: investigating the role of serum ferritin level as a diagnostic tool.

作者信息

Chowdhury Romana, Iktidar Mohammad Azmain, Ahmed Mushfiq Newaz, Hasan Mohammad Mehedi, Tapan Md Mazharul Hoque, Shaheen Sheikh Saiful Islam, Rahman Atiar, Khatun Ayesha

机构信息

Sirajul Islam Medical College, Dhaka, Bangladesh.

North South University, Dhaka, Bangladesh; Public Health Professional Development Society (PPDS), Dhaka, Bangladesh; Director General Health Services (DGHS), Bangladesh.

出版信息

Hematol Transfus Cell Ther. 2023 Jul-Sep;45(3):350-357. doi: 10.1016/j.htct.2022.06.010. Epub 2022 Aug 11.

Abstract

INTRODUCTION

Hypogonadism is one of the most frequent complications in transfusion-dependent thalassemia patients and early recognition and treatment is the core element in restoring impaired gonadal function. Despite the high burden of disease, relevant studies are scarcely addressing the gonadal function of such patients in Bangladesh. The pattern of gonadal function in transfusion-dependent thalassemia patients must be characterized before planning a generalized management plan. Moreover, since iron overload is a key reason behind hypogonadism in thalassemia patients, investigating the role of serum ferritin level as a diagnostic tool for hypongadism was also an aim of this study.

METHODS

This cross-sectional study was conducted at the Department of Transfusion Medicine of the Bangabandhu Sheikh Mujib Medical University. According to the inclusion and exclusion criteria, a total of 94 patients were enrolled in this study. A detailed history and thorough clinical examination were carried out in each patient and recorded using a pretested structured questionnaire. In addition, the laboratory assessment of serum ferritin, luteinizing hormone (LH), follicle stimulating hormone (FSH), testosterone and estradiol in serum were also performed. The data were analyzed using the STATA (v.16).

RESULTS

The mean age of the patients with transfusion-dependent thalassemia was 18.81 ± 4.65 (SD), with 53.3% of the patients being male. The overall prevalence of hypogonadism was 35.11%, 18.1% being normogonadotropic, 11.7% being hypogonadotropic and 5.3% being hypergonadotropic. The serum ferritin level was significantly higher (p < 0.001) in patients with hypogonadism (Eugonadal: 2,174.79 (± 749.12) ng/ml; Hypogonadal: 3,572.59 (± 1,199.49) ng/ml). The area under the receiver operating characteristic (ROC) curve of serum ferritin was high (0.83) and the p-value was highly significant (< 0.001).

CONCLUSION

Therefore, the serum ferritin level and gonadal hormone analysis of transfusion-dependent thalassemia patients can be considered a screening tool for assessing gonadal function and early detection and prevention of hypogonadism.

摘要

引言

性腺功能减退是依赖输血的地中海贫血患者最常见的并发症之一,早期识别和治疗是恢复受损性腺功能的核心要素。尽管疾病负担沉重,但在孟加拉国,相关研究很少涉及此类患者的性腺功能。在制定通用管理计划之前,必须明确依赖输血的地中海贫血患者的性腺功能模式。此外,由于铁过载是地中海贫血患者性腺功能减退的关键原因,因此本研究的另一个目的是调查血清铁蛋白水平作为性腺功能减退诊断工具的作用。

方法

本横断面研究在孟加拉国谢赫穆吉布医学大学输血医学系进行。根据纳入和排除标准,本研究共纳入94例患者。对每位患者进行了详细的病史询问和全面的临床检查,并使用预先测试的结构化问卷进行记录。此外,还对血清铁蛋白、黄体生成素(LH)、卵泡刺激素(FSH)、睾酮和雌二醇进行了实验室评估。数据使用STATA(v.16)进行分析。

结果

依赖输血的地中海贫血患者的平均年龄为18.81±4.65(标准差),其中53.3%为男性。性腺功能减退的总体患病率为35.11%,其中18.1%为正常促性腺激素型,11.7%为低促性腺激素型,5.3%为高促性腺激素型。性腺功能减退患者的血清铁蛋白水平显著更高(p<0.001)(性腺功能正常者:2,174.79(±749.12)ng/ml;性腺功能减退者:3,572.59(±1,199.49)ng/ml)。血清铁蛋白的受试者工作特征(ROC)曲线下面积较高(0.83),p值非常显著(<0.001)。

结论

因此,依赖输血的地中海贫血患者的血清铁蛋白水平和性腺激素分析可被视为评估性腺功能以及早期发现和预防性腺功能减退的筛查工具。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/71e6/10499579/d16928bfaf56/gr1.jpg

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