摘要
目的 探讨血清肝纤维化指标透明质酸 (HA)、Ⅲ型前胶原 (PCⅢ )、Ⅳ型胶原 (CⅣ )和层粘蛋白 (LN)在重型β地中海贫血 (简称 β地贫 )肝纤维化诊断中的价值。 方法 检测 4 9例重型 β地贫患儿和 4 1例正常小儿血清HA、PCⅢ、CⅣ、LN水平 ,其中 4 5例地贫患儿行肝组织学检查 ,进行肝纤维化的对比研究。结果 β地贫组血清HA、PCⅢ、CⅣ、LN四项指标水平均显著高于正常小儿组 (P <0 0 1) ,HA、PCⅢ水平与纤维化分期呈低度正相关 (r=0 379、0 4 5 5 ,P均 <0 0 5 ) ,CⅣ及LN水平与纤维化分期无显著性相关 (r=0 312、0 310 ,P均 >0 0 5 )。四项指标联合诊断肝纤维化的判别函数为 :0 0 0 2HA +0 0 0 3PCⅢ +0 0 0 2CⅣ +0 0 0 6LN - 1 85 9。判断的准确率为 82 16 % ,诊断的灵敏度、特异度、阳性预测价值和阴性预测价值分别为 93 88%、6 8 2 9%、77 97%和 90 32 %。HA、PCⅢ水平的升高与肝脏铁含量有一定的相关性 (r =0 342、0 30 5 ,P均 <0 0 5 )。结论 血清HA、PCⅢ在重型 β地贫肝纤维化诊断中的价值高于CⅣ及LN ,四项指标联合检测可提高临床诊断的准确性和可靠性。
Objective The presence of liver fibrosis in patients with beta-thalassemia major has been demonstrated to be an important negative prognostic factor. Identification of liver fibrosis in early stage would be of great value. Hyaluronic acid (HA) , type Ⅲ pre-collagen (PCⅢ), collagen Ⅳ (CⅣ) and laminin (LN) as serum markers were widely used in the diagnosis of liver fibrosis in patients with chronic viral infections or alcoholic liver diseases. However, their values in thalassemic liver fibrosis have not been studied. This work was to determine the serum HA, PCⅢ, CⅣ and LN levels in children with beta-thalassemia major and evaluate the diagnostic utility. Method Serum HA, PCⅢ, CⅣ and LN in 49 hospitalized children with beta-thalassemia major (aged 1-15 years with the media age of 6.27 years) and 41 healthy children served as controls (aged 1-13 years with media age of 6.40 years) were detected by radioimmunoassay (RIA). Forty-five of 49 cases were performed percutaneous liver biopsies, and the histopathological fibrosis was compared with the four serum markers. The correlation and discriminate analysis were used. Results All the serum levels of HA, PCⅢ, CⅣ and LN in beta-thalassemia were significantly higher than those in controls ( P <0.01). In 36 of 45 cases, the histopathology showed liver fibrosis including stageⅠand stageⅡ by biopsies with a positive rate of 80%. The serum levels of four markers increased successively with the aggravation of liver fibrosis from stage 0 to stageⅡ, and significant correlation was observed between the level of HA or PCⅢ and the stage of fibrosis (HA, r =0.379, P =0.017; PCⅢ, r =0.455, P =0.04). While there was no difference between the level of CⅣ or LN and fibrosis (CⅣ, r = 0.312, P =0.053; LN, r =0.310, P =0.055). Using discriminate analysis, the discriminate function of co-detection of the four markers for the diagnosis of fibrosis was 0.002 HA+0.003PCⅢ+0.002CⅣ+0.006LN-1.859, which had a sensitivity of 93.88%, specificity of 68.29%, predictive value of positive test and negative test of 77.97% and 90.32%, respectively. Moreover, there was a significant correlation between the serum level of HA or PCⅢ and the liver iron concentration (HA, r = 0.318, P =0.035; PCⅢ, r =0.305, P =0.044). Conclusion The results suggest that, in beta-thalassemia major with chronic liver disease, HA and PCⅢ showed more practical value in diagnosing liver fibrosis than the levels of CⅣ and LN. The combination of the four serum markers could improve the accuracy and reliability of the diagnosis. A validation study is necessary before introducing into the prediction function during the clinical practice.
出处
《中华儿科杂志》
CAS
CSCD
北大核心
2003年第8期603-606,共4页
Chinese Journal of Pediatrics
基金
美国中华医学基金会 (CMB)资助 (9663 0 )
