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槟城医院依赖输血的地中海贫血症中的内分泌并发症患病率:一项初步研究。

Prevalence of endocrine complications in transfusion dependent thalassemia in Hospital Pulau Pinang: A pilot study.

机构信息

Hospital Pulau Pinang, Department of Medicine, Malaysia.

Hospital Pulau Pinang, Department of Medicine, Endocrine Unit, Malaysia.

出版信息

Med J Malaysia. 2020 Jan;75(1):33-37.

Abstract

INTRODUCTION

Frequent blood transfusions results in iron overload and lead to multiple endocrine complications. In spite of improvements in iron chelation therapy, a significant number of transfusion dependent thalassaemia (TDT) patients still develop endocrine complications. The aim of this study is to evaluate the prevalence of various endocrine complications in our adult TDT patients and to study the correlation with serum ferritin and liver iron concentration (LIC).

METHODS

A retrospective review of all TDT patients treated in Haematology Unit, Hospital Pulau Pinang (HPP) was conducted.

RESULTS

Of the 45 adult TDT patients, 22 were males and 23 were females with mean age of 28.8±6.9 years old. Majority of TDT in HPP were beta thalassemia major (71.1%), followed by E-Beta thalassemia (24.4%) and HbH-Constant Spring (4.4%). Frequency of transfusion was 3-4 weekly. 40.0% of adult TDT suffered from at least one endocrine complication. Among the adult TDT patients with endocrine complication, 50% have one endocrinopathy, 38.9% with two types of endocrinopathies and 11.1% of them have three or more types of endocrinopathies. Hypogonadism (22.2%) was the commonest endocrine complication, followed by osteoporosis (20%), hypothyroidism (13.3%), diabetes mellitus (6.7%) and hypocortisolism (4.4%). Patients with endocrine complications were significantly older. Mean serum ferritin level and LIC was higher among patients with endocrine complications but both were not statistically significant.

CONCLUSION

Endocrinopathy is still prevalent in 40% of adult TDT patients. This leads to higher health-care resource utilization, cost and significant morbidities among patients with TDT. Therefore, regular monitoring and early detection with intensification of chelation therapy is essential.

摘要

简介

频繁输血会导致铁过载,从而引发多种内分泌并发症。尽管铁螯合疗法有所改善,但仍有相当数量的输血依赖型地中海贫血(TDT)患者会出现内分泌并发症。本研究旨在评估我们的成年 TDT 患者中各种内分泌并发症的患病率,并研究其与血清铁蛋白和肝脏铁浓度(LIC)的相关性。

方法

对槟榔屿医院血液科治疗的所有 TDT 患者进行回顾性研究。

结果

45 例成年 TDT 患者中,男性 22 例,女性 23 例,平均年龄 28.8±6.9 岁。槟榔屿医院 TDT 患者中,β地中海贫血重型(71.1%)最多,其次是 E-β地中海贫血(24.4%)和 HbH-Constant Spring(4.4%)。输血频率为每周 3-4 次。40.0%的成年 TDT 患者至少患有一种内分泌并发症。在患有内分泌并发症的成年 TDT 患者中,50%患有一种内分泌疾病,38.9%患有两种类型的内分泌疾病,11.1%的患者患有三种或更多种类型的内分泌疾病。性腺功能减退症(22.2%)是最常见的内分泌并发症,其次是骨质疏松症(20%)、甲状腺功能减退症(13.3%)、糖尿病(6.7%)和皮质醇功能减退症(4.4%)。患有内分泌并发症的患者年龄明显较大。患有内分泌并发症的患者血清铁蛋白水平和 LIC 较高,但均无统计学意义。

结论

内分泌疾病在 40%的成年 TDT 患者中仍然很常见。这导致 TDT 患者的医疗资源利用、成本和发病率显著增加。因此,定期监测和早期检测,并强化螯合治疗至关重要。

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