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目的探讨重型β地中海贫血肝铁负荷与血清铁蛋白和股骨头红骨髓影像学面积的相关性,旨在寻求一个无创和便于动态评估机体铁负荷的方法,为正确指导去铁治疗和造血干细胞移植寻求新途径。方法对1999—2005年的65例长期在中山大学附二医院输血治疗并追踪观察的重型β地中海贫血患儿病例进行回顾性分析。比较65例重型β地中海贫血患儿肝纤维化程度、含铁血黄素沉积分级在年龄、性别上的差异,以及与股骨头近端红骨髓影像学面积和铁蛋白间的相关性。结果中国广东地区重型β地中海贫血患儿铁蛋白随肝纤维化程度递增,各纤维化程度在年龄和性别上差异无显著性。股骨头红骨髓影像学面积与肝纤维化程度相关系数为0.70,P<0.05。结论广东地区重型β地中海贫血发展为肝纤维化的平均年限为6.7年;股骨头红骨髓影像学面积可作为动态、准确和无创评估铁负荷的新方法。
Objective To investigate the correlation between the hepatic iron load in severe β-thalassemia and the imaging area of serum ferritin and red bone marrow of femoral head in order to seek a noninvasive and dynamic method for assessing the body iron load. In order to correctly guide the iron-removing therapy and hematopoietic stem cell transplantation Seek new ways. Methods A total of 65 children with severe β-thalassemia who were transfused from the Second Affiliated Hospital of Sun Yat-sen University from 1999 to 2005 were retrospectively analyzed. To compare the degree of hepatic fibrosis, hemosiderin deposition grade in 65 children with β-thalassemia major and age, gender, and the correlation between the red bone marrow imaging area and ferritin in the proximal femoral head. Results The levels of ferritin in children with severe β-thalassemia in Guangdong Province of China increased with the degree of liver fibrosis. There was no significant difference in age and gender in the degree of fibrosis. The correlation coefficient of femoral head red bone marrow imaging area and degree of liver fibrosis was 0.70, P <0.05. Conclusions The average age of development of severe β-thalassemia in Guangdong is 6.7 years. The area of femoral head red bone marrow can be used as a new method to evaluate the iron load dynamically, accurately and noninvasively.