影响苯丙酮尿症患儿生活质量的相关因素及干预措施

Related factors affecting quality of life of children with phenylketonuria and intervention measures

目的探讨苯丙酮尿症(PKU)患儿生活质量的影响因素。方法选取2014年12月～2016年12月唐山市妇幼保健院收治的76例PKU患儿作为研究组,采用生活质量综合评定量表(GQOLI-74)对其生活质量进行评价,并根据GQOLI-74评分将研究组患儿分为高分组和低分组;选取既往调查的84名河北省在校健康儿童作为对照组。分析PKU患儿生活质量的影响因素。结果研究组GQOLI-74各维度评分及总分明显低于对照组(P <0.05);采用GQOLI-74评分的76例PKU患儿中,高分组54例,低分组22例,两组年龄、监护人文化程度、监护人职业、PKU患儿生活质量比较,差异有统计学意义(P <0.05)。多因素Logistic回归分析发现,PKU患儿生活质量的影响因素为年龄4～10岁(OR=5.370,95%CI:1.861～15.496,P <0.05),保护因素为监护人文化程度(OR=0.288,95%CI:0.094～0.882,P <0.05)、职业为企业/事业单位(OR=0.215,95%CI:0.062～0.746,P <0.05)、其他职业(OR=0.402,95%CI:0.219～0.738,P <0.05)。结论 PKU患儿整体生活质量较健康儿童差,年龄、监护人文化程度、职业为其主要影响因素,应给予针对性干预措施,以提升患儿生活质量。

Objective To explore the influencing factors of quality of life in children with phenylketonuria (PKU). Methods A total of 76 children with PKU admitted to the Tangshan Maternal and Child Health Hospital from December 2014 to December 2016 were selected as the study group, and their quality of life was evaluated by Greneric Quarity of Life Inventor-74 (GQOLI-74). According to GQOLI-74, the children in the study group were divided into high score group and low score group. Eighty-four healthy children in Hebei Province were selected as the control group. The influencing factors of quality of life in children with PKU were analyzed. Results The GQOLI-74 scores of all dimensions and total scores in the study group were significantly lower than those in the control group (P < 0.05). A total of 76 children with PKU were scored by GQOLI-74, 54 cases in high score group and 22 cases in low score group. There were significant differences in age, guardian′s educational level and guardian′s occupation between the two groups (P < 0.05). Multivariate Logistic regression analysis found that the factors influencing the quality of life of children with PKU was age 4-10 years (OR = 5.370, 95% CI: 1.861-15.496, P < 0.05), the protection factor were the guardian′s education level (OR = 0.288, 95% CI: 0.094-0.882, P < 0.05), and the occupation were the enterprise/institution (OR = 0.215, 95% CI: 0.062-0.746, P < 0.05), other occupations (OR = 0.402, 95% CI: 0.219-0.738, P < 0.05). Conclusion The overall quality of life of children with PKU is worse than that of healthy children. Age, the guardian′s education level and occupation are the main influencing factors. Targeted intervention measures should be given to improve the quality of life of children.