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输血依赖性β地中海贫血患儿,体内大量铁积聚导致肝脏功能、心脏功能及内分泌器官功能异常。铁积累来源于反复输血和胃肠道吸收增加。作者用去铁敏对病人进行了前瞻性治疗研究。临床资料作者选择16例3岁～17岁以前未用去铁敏治疗的β地中海贫血患儿作研究对象。每个病儿均作体格发育检查(身高、体重、肝脾大小等)、辅助检查(全血细胞计数、血清铁蛋白、血清铁、铁结合力、丙氨酸转移酶、空腹血糖、凝血酶原时间、24小时尿铁排泄量、胸部摄片、心电图、M型超声、肝活检等)。治疗原则:每日1 g去铁敏,连续1周,然后根据情况每周加1次量直至维持量。除有明显心脏疾病者外,均每日口服维生素C 100mg。并主张低铁饮食和饮茶,以减少铁吸收。治疗期间的每次
In children with transfusion-dependent beta-thalassemia major accumulations of iron in the body lead to liver, cardiac, and endocrine dysfunction. Iron accumulation comes from repeated blood transfusion and increased gastrointestinal absorption. The authors used deferoxamine to prospectively study patients. Clinical data The authors selected 16 cases of children with beta thalassemia who did not receive deferoxamine before the age of 3 to 17 years old. Each sick child was examined for physical development (height, weight, liver and spleen size, etc.), laboratory examinations (complete blood count, serum ferritin, serum iron, iron binding capacity, alanine aminotransferase, fasting blood glucose, prothrombin Time, 24-hour urinary excretion, chest radiography, electrocardiogram, M-mode ultrasound, liver biopsy, etc.). Treatment principles: 1 g go to the daily sensitive to iron, for 1 week, then according to the situation once a week to increase the amount of volume. In addition to obvious heart disease, were daily oral vitamin C 100mg. And advocate low-iron diet and tea, in order to reduce iron absorption. Every time during treatment