先天性心脏病肺动脉高压患儿血浆降钙素基因相关肽水平和基因测序的临床意义  

The clinical significance of calcitonin gene related peptide(CGRP) level and gene sequence in congenital heart disease with pulmonary hypertension

作　　者：张泽伟[1] 叶璟[2] 李建华[1] 姜力骏[1] 高展[1] 

机构地区：[1]浙江大学医学院附属儿童医院心胸外科 [2]温州医学院附属育英儿童医院,温州325027

出　　处：《中华急诊医学杂志》2006年第3期236-238,共3页Chinese Journal of Emergency Medicine

基　　金：国家"十五"攻关资助项目(2002BA709B09);浙江省科技计划资助项目(001110441)

摘　　要：目的探讨先天性心脏病患儿血浆降钙素基因相关肽(CGRP)水平与肺动脉高压的关系和基因序列检测的临床意义。方法使用放射免疫法测定36例左向右分流先天性心脏病患儿血浆CGRP浓度,心脏超声脉冲Doppler术前测肺体动脉收缩压比值(PP/PS),其中29例合并不同程度肺动脉高压,并用直接测序法对上述患儿CGRP基因第5外显子进行测序。结果轻度肺动脉高压患儿血浆CGRP水平低于对照组(P<0.01),中、重度肺动脉高压患儿明显低于对照组(P<0.001)和肺动脉压力正常组(P<0.05)。先天性心脏病患儿血浆CGRP水平与PP/PS呈负相关。36例先天性心脏病患儿和对照组CGRP基因第5外显子直接测序结果未发现有碱基突变现象。结论血浆CGRP水平失衡在先天性心脏病肺动脉高压的发生机制中起重要作用。肺动脉高压患儿CGRP基因在编码序列上未发现存在多态现象。Objective To investigate the clinical significance of CGRP level and gene sequence in congenital heart disease （CHD） with pulmonary hypertension. Methods The level of serum CGRP was tested by radioimmunoassy in 36 children with left to right shunt CHD, including 29 children with different severity of pulmonany hypertension （PH）. Echo cardiography pulse wave Doppler tested the ratio of the pulmonary and systemic arterial pressure. The exon 5 in CGRP gene was directly sequenced in these patients. Results There were significant differences in serum levels of CGRP between the patients with mild PH and control group. The levels of serum CGRP were significantly decreased in patients with moderate or severe PH. There was significant negative llne correlation between the levels of CGRP and pulmonary pressure. Base mutation were not found in the exon 5 in CGRP gene. Conclusion The CGRP might be involved in the pathophysiologic process of pulmonary hypertension in CHD. There is no gene expression polymorphism in encode sequence in CHD patients with pulmonary hypertension.

关 键 词：降钙素基因相关肽 基因多态性 肺动脉高压 心脏缺损/先天性 

分 类 号：R725.4[医药卫生—儿科]