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目的:了解本中心中间型β地中海贫血(β地贫)患者的治疗和并发症现状,探讨如何提高中间型β地贫患者的生存质量。方法:回顾性总结解放军第303医院2011-2014年收治的92例中间型β地贫患者的临床资料,调查患者的临床特征、治疗现状、并发症现状,分析其影响因素。结果:92例患者中,男51例(55.4%),女41例(44.6%),平均年龄(24.9±9.2)岁,起病年龄(4.0±5.8)岁(1~30岁)。91例患者接受过输血治疗(其中15例为规律输血),接受输血者平均血红蛋白为(58.1±20.1)g/L。72例患者有铁超载,但仅22例有接受去铁治疗史。平均血清铁蛋白为(2 172.0±3 728.1)(344~12 482)ng/ml,40例(43.5%)为重度铁超载者(血清铁蛋白>2 500ng/ml)。骨质疏松、肺动脉高压、肝功能异常、胆囊结石、髓外造血的发生比例分别为54.1%、55.7%、43.3%、44.1%和9.7%。结论:广西地区本中心中间型β地贫患者的治疗很不充分,并发症发生率高。需加强对中间型β地贫患者的输血和去铁治疗,以提高患者生存质量。
OBJECTIVE: To understand the treatment and complications of intermediate β-thalassemia (β-thalassemia) in our center and to explore ways to improve the quality of life in patients with intermediate β-thalassemia. Methods: The clinical data of 92 patients with intermediate type β thalassemia admitted to the 303 Hospital of PLA from 2011 to 2014 were retrospectively reviewed. The clinical features, treatment status and complications of the patients were investigated. The influencing factors were analyzed. Results: Of the 92 patients, 51 (55.4%) were male and 41 (44.6%) were female, with an average age of 24.9 ± 9.2 years and a mean age of onset of 4.0 ± 5.8 years (range 1 to 30 years). Ninety-one patients received blood transfusions (15 of whom were regular transfusions) and a mean of 58.1 ± 20.1 g / L of blood transfusion recipients. 72 patients had iron overload, but only 22 had a history of deferox treatment. The mean serum ferritin was (2 172.0 ± 3 728.1) (344-12482) ng / ml and 40 (43.5%) were severe iron overload (serum ferritin> 2500ng / ml). Osteoporosis, pulmonary hypertension, abnormal liver function, gallstone, and extramedullary hematopoiesis were 54.1%, 55.7%, 43.3%, 44.1% and 9.7%, respectively. Conclusion: The treatment of intermediate β-thalassemia patients in Guangxi is inadequate and the complication rate is high. It is necessary to strengthen the blood transfusion and iron-removing therapy for patients with β-thalassemia intermediate in order to improve the quality of life of patients.