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感染与地中海贫血

Infections and thalassaemia.

作者信息

Vento Sandro, Cainelli Francesca, Cesario Francesco

机构信息

Section of Infectious Diseases, Department of Pathology, University of Verona, Verona, Italy.

出版信息

Lancet Infect Dis. 2006 Apr;6(4):226-33. doi: 10.1016/S1473-3099(06)70437-6.

Abstract

Infections are major complications and constitute the second most common cause of mortality and a main cause of morbidity in patients with thalassaemia, a group of genetic disorders of haemoglobin synthesis characterised by a disturbance of globin chain production. Thalassaemias are among the most common genetic disorders in the world. Predisposing factors for infections in thalassaemic patients include severe anaemia, iron overload, splenectomy, and a range of immune abnormalities. Major causative organisms of bacterial infections in thalassaemic patients are Klebsiella spp in Asia and Yersinia enterocolitica in western countries. Transfusion-associated viral infections (especially hepatitis C) can lead to liver cirrhosis and hepatocellular carcinoma. A unique and challenging infection detected in Asian patients is pythiosis, caused by a fungus-like organism, the mortality rate of which is very high. Because the prognosis for thalassaemia has much improved, with many patients surviving to the fifth decade of life in developed countries, it is mandatory to reduce mortality by recognising and presumptively treating infections in these patients as quickly as possible.

摘要

感染是主要并发症,是地中海贫血患者死亡的第二大常见原因和发病的主要原因,地中海贫血是一组血红蛋白合成的遗传性疾病,其特征是珠蛋白链生成紊乱。地中海贫血是世界上最常见的遗传性疾病之一。地中海贫血患者感染的易感因素包括严重贫血、铁过载、脾切除术和一系列免疫异常。亚洲地中海贫血患者细菌感染的主要致病菌是克雷伯菌属,西方国家是小肠结肠炎耶尔森菌。输血相关的病毒感染(尤其是丙型肝炎)可导致肝硬化和肝细胞癌。在亚洲患者中检测到的一种独特且具有挑战性的感染是由一种类真菌生物引起的腐皮病,其死亡率非常高。由于地中海贫血的预后有了很大改善,在发达国家许多患者存活到了五十岁,因此必须通过尽快识别并对这些患者的感染进行经验性治疗来降低死亡率。

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