摘要
目的探讨代谢控制联合四氢生物蝶呤对早期苯丙酮尿症患儿生活质量的影响。方法选取该院2016年12月至2018年6月收治的76例苯丙酮尿症患儿,按照随机数字表法分为对照组和观察组,各38例,对比干预后患儿身高、体质量、智能发育以及生活质量的变化。结果两组患儿干预后的身高、体质量对比,差异无统计学意义(P>0.05),随干预的时间延长,两组患儿身高、体质量不断增加,与治疗前比较,差异均有统计学意义(P<0.05)。干预后,观察组苯丙氨酸水平明显低于对照组,差异有统计学意义(P<0.05)。观察组患儿未出现临床症状,临床治疗效果较好,智能发育达到正常水平,且优于对照组。观察组生活质量各项指标评分优于对照组,差异均有统计学意义(P<0.05)。结论对早期苯丙酮尿症患儿实施代谢控制联合四氢生物蝶呤干预治疗,能够显著降低患儿苯丙氨酸水平,改善患儿的生活质量,促进患儿智力恢复。
Objective To discuss the effect of metabolic control combined with tetrahydrobiopterin on the quality of life in children patients with early phenylketonuria(PKU).Methods Seventy-six children patients with PKU in this hospital from December 2016 to June 2018 were selected and divided into the control group and the observation group according to the random number table method,38 cases in each group.The changes of the height,body mass,intellectual development and quality of life after intervention were compared between the two groups.Results After intervention,there was no statistically significant difference in height and body mass between the two groups(P>0.05).With the extension of intervention time,the height and body mass were increased continuously,and the difference compared with before treatment was statistically significant(P<0.05).After the intervention,the level of phenylalanine(Phe)in the observation group was significantly lower than that in the control group,and the difference was statistically significant(P<0.05).The children patients in the observation group showed no clinical symptoms,the clinical treatment effect was good,and the intelligence development reached the normal level,which were higher than those in the control group.The scores of living quality indicators in the observation group were all better than those in the control group,and the differences were statistically significant(P<0.05).Conclusion Implementing the metabolic control combined with tetrahydroxopterin intervention in early phenylketonuria can significantly reduce Phe level,improve the quality of life and promote the intellectual development in children patients.
作者
韩宗兰
王兰英
王海楠
陈娜
于静
HAN Zonglan;WANG Lanying;WANG Hainan;CHEN Na;YU Jing(Tangshan Municipal Maternal and Child Health Care Hospital,Tangshan,Hebei 063000,China)
出处
《检验医学与临床》
CAS
2019年第11期1491-1494,共4页
Laboratory Medicine and Clinic
基金
河北省卫生和计划生育委员会科研基金项目(20181328)
关键词
代谢控制
四氢生物蝶呤
生活质量
苯丙酮尿症
metabolic control
tetrahydrobiopterin
quality of life
phenylketonuria
