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目的 探讨重型β-地中海贫血患儿肝脏病理改变特点及其与临床的关系,评价肝活检病理对治疗指导的意义。方法 对22例重型β-地中海贫血患儿行肝活检病理检查,并结合临床资料进行分析。结果 22例穿刺均1次成功,肝组织有不同程度的铁沉积,沉积程度与输血量及血清铁蛋白相关。汇管区纤维组织增生,纤维化程度与铁沉积程度无相关。结论 我国重型β-地中海贫血患儿肝病理变化的特点是铁沉积重,纤维化程度相对轻。小儿肝穿刺活检是安全、可行的。
Objective To investigate the pathological changes of liver in children with β-thalassemia major and its relationship with clinic, and to evaluate the significance of liver biopsy pathology in treatment guidance. Methods Twenty-two children with severe β-thalassemia were examined by liver biopsy and analyzed with clinical data. Results All the 22 cases were successfully punctured. There was different degree of iron deposition in the liver tissue. The degree of deposition was related to the amount of blood transfusion and serum ferritin. There was no correlation between fibrous tissue proliferation and fibrosis in portal area. Conclusion The pathological changes of liver in children with β-thalassemia major are characterized by heavy iron deposition and relatively low degree of fibrosis. Pediatric liver biopsy is safe and feasible.