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地中海贫血患者的登革出血热

Dengue hemorrhagic fever in patients with thalassemia.

作者信息

Pongtanakul Bunchoo, Narkbunnam Nattee, Veerakul Gavivann, Sanpakit Kleebsabai, Viprakasit Vip, Tanphaichitr Voravarn Tanphaichitr, Suvatte Vinai

机构信息

Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand.

出版信息

J Med Assoc Thai. 2005 Nov;88 Suppl 8:S80-5.

Abstract

Dengue hemorrhagic fever (DHF) causing by dengue viral infection is endemic in Thailand and Southeast Asian countries where thalassemias are prevalent. Thalassemic patients are also at risk to acquire dengue viral infections and to develop DHF. However, they can have different clinical manifestations and complications as well as more severity than general population requiring special awareness for proper diagnosis and management. We reported 20 thalassemic patients (10 boys and 10 girls) with DHF admitted to Department of Pediatrics, Siriraj Hospital during 1977 to 2001. Their ages ranged from 2-16 years (average 9.5 years). These cases included 5 cases of Hb H disease, 5 cases of Hb H with Hb Constant Spring (CS), 9 cases of beta-thalassemia/Hb E disease and 1 case of beta-thalassemia major. Two cases were in Grade I, 10 cases in grade II, 7 cases in Grade III and one case in grade IV severity of DHF. Though there were evidences of plasma leakage, instead of hemoconcentration, eighteen patients (90 percent) had hematocrit dropped at the range of 11-66% of the initial level. Fifteen patients (75 percent) required at least one packed red cell transfusion. Nine patients (45 percent) had mild bleeding symptoms, one of them had upper gastrointestinal hemorrhage requiring platelet concentrate transfusion. Two patients (10 percent) had serious complications including one with infection-associated hemophagocytic syndrome (IAHS) requiring intravenous immunoglobulin (IVIG) and packed red cell transfusion and the other had generalized seizure due to hyponatremia and hypotension. No mortality was observed among this group of patients. Early recognition of the DHF in thalassemic patients and appropriate packed red cell transfusion in patients with anemic symptoms is warranted to reduce morbidity and mortality in these patients.

摘要

由登革病毒感染引起的登革出血热(DHF)在泰国和东南亚国家呈地方性流行,这些地区地中海贫血症也很普遍。地中海贫血症患者也有感染登革病毒并发展为登革出血热的风险。然而,他们可能有不同的临床表现和并发症,且比普通人群病情更严重,需要特别关注以进行正确的诊断和管理。我们报告了1977年至2001年期间在诗里拉吉医院儿科收治的20例患有登革出血热的地中海贫血症患者(10名男孩和10名女孩)。他们的年龄在2至16岁之间(平均9.5岁)。这些病例包括5例血红蛋白H病、5例血红蛋白H合并血红蛋白恒河猴(CS)、9例β地中海贫血/血红蛋白E病和1例重型β地中海贫血。2例为I级登革出血热,10例为II级,7例为III级,1例为IV级。尽管有血浆渗漏的证据,但与血液浓缩不同,18例患者(90%)的血细胞比容下降至初始水平的11%至66%。15例患者(75%)至少需要输注一次浓缩红细胞。9例患者(45%)有轻度出血症状,其中1例有上消化道出血,需要输注血小板浓缩液。2例患者(10%)出现严重并发症,其中1例患有感染相关噬血细胞综合征(IAHS),需要静脉注射免疫球蛋白(IVIG)和输注浓缩红细胞,另1例因低钠血症和低血压出现全身性惊厥。该组患者中未观察到死亡病例。早期识别地中海贫血症患者的登革出血热,并对有贫血症状的患者进行适当的浓缩红细胞输注,对于降低这些患者的发病率和死亡率是必要的。

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