摘要
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 hepatic tissue pathological features and its relationship with clinical data in children with β-thalassemia major, and to evaluate the value of liver biopsy in appropriate treament before hematopoietic stem cell transplantation. Methods 22 hopitalized thalassemic patients (ranged 2-10 years) from 1998 to 2000underwent percutaneous liver biopsies with ultrasound guidance and under anaesthesia. Pathological and clinical analysis were made. Results All biopsies were successfully performed by only once. Liver iron deposits were relatively severe and significantly correlated with serum ferritin (r=0- 65 P<0. 05) and the volume of blood transfusion (r=0. 63 P<0. 05) respectively. Liver fibrosis, however, were mild, and no correlation was observed between fibrosis and iron deposition ( P>0. 05). Conclusion Severe liver iron loading but mild fibrosis was the main pathological characteristic of liver in children with β-thalassemia major in our country. Being well controlled, liver biopsy in children is safe and feasible.
出处
《中国优生优育（1990-2002上半年）》
2001年第3期123-125,130,共4页
Journal of Improving Birth Outcome and Child Development of China
