目的 探讨先天性心脏病(CHD)相关性肺动脉高压(PAH)患儿血浆内源性二氧化硫(SO2)、同型半胱氨酸(Hcy)的水平及其与肺动脉压力之间的关系,并分析其在疾病中的作用.方法 采用前瞻性队列研究,对2012年7月至2013年10月在兰州大学第二医院儿科及心脏外科就诊的左向右分流型CHD患儿,按PAH-CHD纳入标准、合并症排除、肺动脉压测定等限定条件分为4组:(1)CHD无PAH组20例,其中男10例、女10例,室间隔缺损(VSD)5例,房间隔缺损(ASD)8例,动脉导管未闭(PDA)7例,年龄1.2(0.8,2.4)岁;(2)CHD轻度PAH组20例,其中男10例、女10例,VSD12例,ASD 6例,PDA 2例,年龄0.7(0.5,1.2)岁;(3) CHD中重度PAH组20例,其中男8例、女12例,VSD 12例,ASD 6例,PDA 1例,VSD+ ASD 1例,年龄0.9(0.6,3.0)岁;(4)另以同期门诊体检的健康儿童20名为对照组,其中男8名、女12名,年龄1.4(0.8,2.9)岁.采用高效液相色谱荧光法,分别测定血浆Hcy与SO2及亚硫酸盐SO2-/HSO3-的含量,运用方差分析进行组间及组内比较,并Pearson双变量分析其相关性.结果 (1)血浆Hcy水平在对照组、CHD无PAH组、CHD轻度PAH组及CHD中重度PAH组中分别为(11.0±2.7)、(11.7±2.5)、(12.0±2.1)和(14.3±3.2) μmol/L;组间多重比较,CHD中重度PAH组最高(均P＜0.05),其他3组之间两两比较,差异均无统计学意义(P均＞0.05).(2)SO2含量(以SO32-计)在对照组、CHD无PAH组、CHD轻度PAH组及CHD中重度PAH组中分别为(10.6±2.4)、(8.9±2.3)、(7.3±2.9)和(4.3±2.1)μmol/L,单因素方差分析及组间多重比较,两两之间差异均有统计学意义(P均＜0.05).(3)在CHD无PAH组中,Hcy水平和SO2含量在VSD、ASD、PDA中差异均无统计学意义(P均＞0.05).(4)VSD与ASD两型的Hcy水平和SO2含量在CHD轻度PAH组和CHD中重度PAH组中,差异也均无统计学意义(均P＞0.05).(5) Pearson相关性分析显示,在CHD各组中Hcy水平与肺动脉压呈正相关(r=0.481,P＜0.01),而SO2含量与肺动脉压呈负相关(r=-0.553,P＜0.01).在4组所有儿童中,Hcy与SO2存在着负相关(r=-0.231,P＜0.05).结论 PAH-CHD患儿有着低SO2、高Hcy的倾向,表明含硫氨基酸代谢通路之一的Hcy-SO2可能存在着异常,Hcy可作为判断严重PAH-CHD的生物学指标之一,而SO2有望成为PAH-CHD治疗的新靶向.
目的 探討先天性心髒病(CHD)相關性肺動脈高壓(PAH)患兒血漿內源性二氧化硫(SO2)、同型半胱氨痠(Hcy)的水平及其與肺動脈壓力之間的關繫,併分析其在疾病中的作用.方法 採用前瞻性隊列研究,對2012年7月至2013年10月在蘭州大學第二醫院兒科及心髒外科就診的左嚮右分流型CHD患兒,按PAH-CHD納入標準、閤併癥排除、肺動脈壓測定等限定條件分為4組:(1)CHD無PAH組20例,其中男10例、女10例,室間隔缺損(VSD)5例,房間隔缺損(ASD)8例,動脈導管未閉(PDA)7例,年齡1.2(0.8,2.4)歲;(2)CHD輕度PAH組20例,其中男10例、女10例,VSD12例,ASD 6例,PDA 2例,年齡0.7(0.5,1.2)歲;(3) CHD中重度PAH組20例,其中男8例、女12例,VSD 12例,ASD 6例,PDA 1例,VSD+ ASD 1例,年齡0.9(0.6,3.0)歲;(4)另以同期門診體檢的健康兒童20名為對照組,其中男8名、女12名,年齡1.4(0.8,2.9)歲.採用高效液相色譜熒光法,分彆測定血漿Hcy與SO2及亞硫痠鹽SO2-/HSO3-的含量,運用方差分析進行組間及組內比較,併Pearson雙變量分析其相關性.結果 (1)血漿Hcy水平在對照組、CHD無PAH組、CHD輕度PAH組及CHD中重度PAH組中分彆為(11.0±2.7)、(11.7±2.5)、(12.0±2.1)和(14.3±3.2) μmol/L;組間多重比較,CHD中重度PAH組最高(均P＜0.05),其他3組之間兩兩比較,差異均無統計學意義(P均＞0.05).(2)SO2含量(以SO32-計)在對照組、CHD無PAH組、CHD輕度PAH組及CHD中重度PAH組中分彆為(10.6±2.4)、(8.9±2.3)、(7.3±2.9)和(4.3±2.1)μmol/L,單因素方差分析及組間多重比較,兩兩之間差異均有統計學意義(P均＜0.05).(3)在CHD無PAH組中,Hcy水平和SO2含量在VSD、ASD、PDA中差異均無統計學意義(P均＞0.05).(4)VSD與ASD兩型的Hcy水平和SO2含量在CHD輕度PAH組和CHD中重度PAH組中,差異也均無統計學意義(均P＞0.05).(5) Pearson相關性分析顯示,在CHD各組中Hcy水平與肺動脈壓呈正相關(r=0.481,P＜0.01),而SO2含量與肺動脈壓呈負相關(r=-0.553,P＜0.01).在4組所有兒童中,Hcy與SO2存在著負相關(r=-0.231,P＜0.05).結論 PAH-CHD患兒有著低SO2、高Hcy的傾嚮,錶明含硫氨基痠代謝通路之一的Hcy-SO2可能存在著異常,Hcy可作為判斷嚴重PAH-CHD的生物學指標之一,而SO2有望成為PAH-CHD治療的新靶嚮.
목적 탐토선천성심장병(CHD)상관성폐동맥고압(PAH)환인혈장내원성이양화류(SO2)、동형반광안산(Hcy)적수평급기여폐동맥압력지간적관계,병분석기재질병중적작용.방법 채용전첨성대렬연구,대2012년7월지2013년10월재란주대학제이의원인과급심장외과취진적좌향우분류형CHD환인,안PAH-CHD납입표준、합병증배제、폐동맥압측정등한정조건분위4조:(1)CHD무PAH조20례,기중남10례、녀10례,실간격결손(VSD)5례,방간격결손(ASD)8례,동맥도관미폐(PDA)7례,년령1.2(0.8,2.4)세;(2)CHD경도PAH조20례,기중남10례、녀10례,VSD12례,ASD 6례,PDA 2례,년령0.7(0.5,1.2)세;(3) CHD중중도PAH조20례,기중남8례、녀12례,VSD 12례,ASD 6례,PDA 1례,VSD+ ASD 1례,년령0.9(0.6,3.0)세;(4)령이동기문진체검적건강인동20명위대조조,기중남8명、녀12명,년령1.4(0.8,2.9)세.채용고효액상색보형광법,분별측정혈장Hcy여SO2급아류산염SO2-/HSO3-적함량,운용방차분석진행조간급조내비교,병Pearson쌍변량분석기상관성.결과 (1)혈장Hcy수평재대조조、CHD무PAH조、CHD경도PAH조급CHD중중도PAH조중분별위(11.0±2.7)、(11.7±2.5)、(12.0±2.1)화(14.3±3.2) μmol/L;조간다중비교,CHD중중도PAH조최고(균P＜0.05),기타3조지간량량비교,차이균무통계학의의(P균＞0.05).(2)SO2함량(이SO32-계)재대조조、CHD무PAH조、CHD경도PAH조급CHD중중도PAH조중분별위(10.6±2.4)、(8.9±2.3)、(7.3±2.9)화(4.3±2.1)μmol/L,단인소방차분석급조간다중비교,량량지간차이균유통계학의의(P균＜0.05).(3)재CHD무PAH조중,Hcy수평화SO2함량재VSD、ASD、PDA중차이균무통계학의의(P균＞0.05).(4)VSD여ASD량형적Hcy수평화SO2함량재CHD경도PAH조화CHD중중도PAH조중,차이야균무통계학의의(균P＞0.05).(5) Pearson상관성분석현시,재CHD각조중Hcy수평여폐동맥압정정상관(r=0.481,P＜0.01),이SO2함량여폐동맥압정부상관(r=-0.553,P＜0.01).재4조소유인동중,Hcy여SO2존재착부상관(r=-0.231,P＜0.05).결론 PAH-CHD환인유착저SO2、고Hcy적경향,표명함류안기산대사통로지일적Hcy-SO2가능존재착이상,Hcy가작위판단엄중PAH-CHD적생물학지표지일,이SO2유망성위PAH-CHD치료적신파향.
Objective To determine the relationship between the serum sulfur dioxide,homocysteine and the pulmonary arterial pressure in children with congenital heart defects who generated a pulmonary arterial hypertension syndrome (PAH-CHD),and analyze their role in the pathological process of the disease.Method This was a prospective cohort study,children with systemic pulmonary shunt CHD were selected.The patients were devided into three groups:the CHD with no PAH group:n =20,10 males,10 females,5 with ventricular septal defect (VSD),8 with atrial septal defect (ASD) and 7 with patent ductus arteriosus (PDA),mean age (1.9 ± 1.8) years; the CHD with mild PAH group:n =20,10 males,10 females,12 with VSD,6 with ASD,and 2 with PDA,mean age (1.0 ± 0.8) year; the CHD with moderate or severe PAH group:n =20,8 males,12 females,12 with VSD,6 with ASD,and 1 with PDA,1 with ASD + VSD,mean age (1.8 ± 1.6) year.Twenty healthy children were enrolled from outpatient department as the control group [included 8 males,12 females,mean age (1.9 ± 1.5) years].The homocysteine and SO2 concentrations in the serum samples were detected by a modified high performance liquid chromatographic method with fluorescence detection (HPLC-FD),then,multiple comparisons among the groups were performed with analysis of variance,and the pearson correlation.Result The serum homocysteine concentrations were respectively (11.0 ± 2.7),(11.7 ± 2.5),(12.0 ± 2.1),(14.3 ± 3.2) μmol/L in the control group,CHD with no PAH group,CHD with mild PAH group,and CHD with moderate or severe PAH group.According to the multiple comparisons,the CHD with moderate or severe PAH group had the highest level(P all ＜ 0.05).While the comparison within the control group,CHD with none PAH group,and CHD with mild PAH group,the differences were not significant(P all ＞ 0.05).The serum sulfur dioxide strength (concentrated asSO32-) were respectively (10.6 ±2.4),(8.9 ±2.3),(7.3 ±2.9),(4.3 ±2.1) μmol/L in the control group,CHD with none PAH group,CHD with mild PAH group,and CHD with moderate or severe PAH group.CHD with moderate or severe PAH group had the highest level of serum sulfur dioxide (P ＜ 0.05).The pearson correlation analysis indicated that in the CHD children,the serum homocysteine were positively correlated with the pulmonary arterial pressure (r =0.481,P ＜ 0.01),while,the sulfur dioxide were negatively correlated with pulmonary arterial pressure (r =-0.553,P ＜ 0.01).In all children,the serum homocysteine levels were negatively correlated with the sulfur dioxide(r =-0.231,P =0.039).Conclusion The PAH-CHD children had higher homocysteine levels and lower sulfur dioxide levelsl,which demonstrated the disturbance of homocysteine-sulfur dioxide pathway in the sulfur containing amino acids metabolish in the disease.The homocysteine may become a biological marker which reflecting the severities of the PAH-CHD,while the sulfur dioxide can be a new target for the therapy of PAH-CHD.
