【摘要】 目的探讨内皮素(ET-1)、C-MYC癌基因在先天性心脏病(CHD)合并肺动脉高压(PH)形成过程中的生物作用。方法42例左向右分流CHD患者,根据肺动脉平均压(MPAP)的高低分为三组,正常组:MPAP<20mmHg,11例;肺动脉高压组:MPAP>20mmHg,22例;重度肺动脉高压组:MPAP>70mmHg,9例。采用放免法测定血浆ET-1浓度。所有病例均行肺活检术,标本常规制成蜡块,应用免疫组化方法,单克隆抗C-MYC基因蛋白抗体作一抗,观察肺组织中C-MYC癌基因的定位及表达。结果C-MYC癌基因主要分布于肺动脉内皮细胞、平滑肌和成纤维细胞等细胞的胞核中。血浆ET-1浓度和C-MYC癌基因表达各组之间差异有统计学意义(P<0.05),各病理分级之间差异有统计学意义(P<0.05),随着肺小动脉病理分级的加重而升高,并且二者的表达与病理分级有明显的相关性(P<0.05)。结论ET-1和C-MYC癌基因在CHD合并PH的形成过程中起着重要的生物学作用,ET-1可以刺激C-MYC癌基因的表达,进而促进平滑肌细胞和成纤维细胞增殖。更多还原

【Abstract】 Objective To study the concentration of plasma ET-1 and expression of C-MYC gene in the pulmonary of patients with congenital heart disease associated with pulmonary hypertension and its significance and the relationship in the concentration of plasma ET-1 and expression of C-MYC gene and pathological behavior of grading and staging of hyper-kinesic pulmonary hypertension. Methods 42 patients with CHD were divided into three groups, no-PH group (11 patients), with preoperative mean pulmonary artery pressure (MPAP) <20 mm Hg; PH group:(22 patients), with preoperative MPAP>20 mm Hg, and cyanosis group (9 patients), with preoperative MPAP>70 mm Hg. The lung tissue samples were examined from 42 patients by surgical biopsy of lung .Lung tissue was examined for expression of C-MYC by immunohistochemical technique.Pathological grading of pulmonary vascular changes was standardized by Heath-Edwards. Radio-immunal assay was used to determine the concentration of plasma ET-1. Results There is expression karyon of endotheliocyte,smooth muscle cell and growth fibrin cell of pulmonary C-MYC gene staining.The concentration of ET-1 and the expression of C-MYC in the lung tissues of PH groups were increased graduslly.The expression of C-MYC in the lung tissues in all pathological degree groups showed statistic differences(P<0.05=. The concentration of plasma ET-1 was correlative with positive expression of c-myc gene(P<0.05=. Conclusion The finding confirmed that ET-1 and C-MYC gene have an important biological behavior in the process of formed pulmonary hypertension of congenital heart disease and the ET-1 could activate expression of C-MYC gene.They improve proliferation of smooth muscle cell and growth fibrin cell.更多还原