摘要
目的:通过检测苯丙酮尿症患儿血液中非对称二甲基精氨酸和苯丙氨酸水平,观察其对患儿临床症状改善相关性,分析对称二甲基精氨酸与精氨酸等因素对苯丙酮尿症患者病情控制的预测价值。方法:选取2016年9月至2019年3月在承德市妇幼保健院定期复查的PKU患儿96例,高效液相色谱法检测血液中苯丙氨酸(Phenylalanine,PHE)浓度,并根据苯丙氨酸控制水平分为控制良好组(Phe240~600μmoL/L)和控制不佳组(Phe>600μmoL/L);另选取100名健康儿童设为对照组。比较三组患儿临床资料、实验室指标采用高效液相色谱法,该检测采用高效液相色谱仪检测对称苯丙氨酸(Phenylalanine,PHE)、二甲基精氨酸(Symmetric dimethylarginine,SDMA)、非对称二甲基精氨酸(Asymmetric dimethylarginine,ADMA)、精氨酸(Arginine,ARG)、总同型半胱氨酸(Totalhomocysteine,HCY)。全自动生化检测仪检测血肌酐(Serum creatinine,SCR)、甘油三酯(Triglyceride,TG)、总胆固醇(Totalcholesterol,TC)等,并记录患儿的临床症状、智力评分等资料;分析ADMA与PHE、智力评分等指标的相关性;多因素logistic回归分析影响患儿智力发育的独立相关因素。结果:与对照组比较,PKU患儿的智力评分、ADMA、SDMA、ARG、HCY水显著低于对照组,有统计学差异(P<0.05);与控制不良组比较,控制良好组患儿的ADMA、SDMA、ARG水平及智力评分显著升高,有统计学差异(P<0.05);ADMA、SDMA、ARG和HCY是PKU患儿苯丙氨酸浓度的主要影响因(P<0.05),其中,ADMA与SDMA,ARG三种因素与苯丙氨酸浓度呈负相关,与智力评分呈正相关,相关系数分别为0.6、0.7、0.4;多因素logistic回归分析显示,高PHE水平(OR=1.952)是影响智力发育的独立相关因素(P<0.05),而年龄越大(OR=1.074)和高ADMA水平(OR=1.556)是患儿智力发育的保护性因素(P<0.05)。结论:ADMA可以作为评估PKU患儿饮食代谢控制的一项新兴指标,且能够有效预测患儿智力发育情况。
Objective:To observe the correlation between the improvement of clinical symptoms and analyze the factors such as symmetric dimethylarginine and arginine by detecting the levels of asymmetric dimethylarginine and phenylalanine in the blood of children with phenylketonuria and to analyze the predictive value of the disease control in patients with phenylketonuria.Methods:Ninety-six children with PKU who were regularly reexamined in Chengde Maternal and Child Health Hospital from September 2016 to March 2019 were selected.The concentration of phenylalanine(Phe)in the blood was detected by high performance liquid chromatography.Patients were divided into a well-controlled group(Phe240~600μmol/L)and a poorly-controlled group(Phe>600μmol/L)considering the control level of phenylalanine;another 100 healthy children were selected and made the control group.The clinical data and laboratory indicators of the three groups of children were compared using high-performance liquid chromatography.The detection covers PHE,symmetric dimethylarginine(SDMA),asymmetric dimethylarginine(ADMA),arginine(ARG),total homocysteine(HCY).Automatic biochemical detector was used to detect serum creatinine(Serum creatinine,SCR)fine Amino acid(Arginine,ARG),triglyceride(TG),total cholesterol(Totalcholesterol,TC),etc.,and the clinical symptoms and intelligence scores of children were recorded;the correlation between ADMA and Phe,intelligence scores and other indicators was analyzed;multifactorial logistic regression analysis of independent related factors affecting children's intelligence development was conducted.Results:Compared with the control group,the intelligence score,ADMA,SDMA,Arg,Hcy water of PKU children were significantly lower than the control group,with statistical differences(P<0.05);compared with the poorly controlled group,the ADMA,SDMA,ARG levels and intelligence scores of the well-controlled group were significantly increased,with statistical differences(P<0.05);DMA,SDMA,ARG and HCY were the main factors affecting the concentration of phenylalanine(P<0.05).Among them,ADMA,SDMA,and ARG were negatively correlated with phenylalanine concentration,and positively correlated with intelligence scores.The correlation coefficients were 0.6,0.7,and 0.4,respectively.Multivariate logistic regression analysis shows that high PHE level(OR=1.952)is an independent related factor that affects intellectual development(P<0.05),while older age(OR=1.074)and high ADMA level(OR=1.556)are protective factors for children's intellectual development(P<0.05).Conclusion:ADMA can be used as an emerging indicator for evaluating dietary metabolism control in children with PKU,and can effectively predict children's intellectual development.
作者
左雪梅
姚淑慧
王宏蕾
陈海荣
杨志敏
张铁群
金艳文
ZUO Xuemei(Chengde Maternal and Child Health Hospital,Hebei Chengde 067000,China)
出处
《河北医学》
CAS
2021年第2期201-206,共6页
Hebei Medicine
基金
河北省承德市科学技术研究与发展计划项目,(编号:202006A006)。
