山西省277例苯丙酮尿症患儿智力影响因素分析  

Influencing factors of intelligence in 277 children with phenylketonuria in Shanxi Province

作　　者：穆文娟 郝丽婷 杨建平 朱玲 MU Wenjuan;HAO Liting;YANG Jianping;ZHU Ling(Shanxi Children′s Hospital,Shanxi Maternal and Child Health Hospital,Shanxi Taiyuan 030013,China)

机构地区：[1]山西省儿童医院山西省妇幼保健院,山西太原030013

出　　处：《中国妇幼健康研究》2023年第3期63-68,共6页Chinese Journal of Woman and Child Health Research

摘　　要：目的 对苯丙酮尿症(PKU)患儿智力发展的可能影响因素进行分析,为制定相关公共政策、开展PKU的健康教育和家庭指导提供支撑。方法 以山西省妇幼保健院新生儿疾病筛查中心于2004年6月至2020年1月筛查并治疗的277例PKU患儿作为研究对象,通过查阅病历、电话随访获取患儿的一般资料及最近1次的智力测试结果、治疗依从性、血苯丙氨酸(Phe)浓度控制情况等,分析影响PKU患儿智力水平的因素。结果 共纳入PKU患儿277例,277例PKU患儿平均智力为(94.10±13.57)分,其中智力水平在中等及以上的有243例,占比87.73%;智力水平为临界偏低和发育障碍的患儿占比12.27%,良好和优秀的患儿占比9.74%。<3岁的患儿(F=8.108)、带养人年龄在25~35岁(F=7.224)、带养人受教育程度在大专及以上(F=44.339)的患儿智力水平相对较高(P<0.05);城镇患儿智力水平高于农村(t=-3.336)、祖辈支持程度较高的患儿智力水平高于支持程度较低的(t=-5.549),患儿开始治疗年龄≤1月龄(t=4.356)、确诊时Phe浓度<20mg/dL(t=2.148)、治疗依从性好(t=-4.130)及血Phe浓度控制情况好(t=-3.335)的患儿其智力水平较高(P<0.05)。多重线性回归分析显示,患儿年龄(β=-0.100)和开始治疗的年龄(β=-0.581)与PKU患儿智力水平呈负相关;家庭类型(β=0.105)、祖辈支持程度(β=0.218)、家庭收入(β=0.173)、治疗依从性(β=0.112)与PKU患儿智力水平呈正相关。对智商水平均数的影响贡献从大到小排序依次是:开始治疗年龄(β=-0.581)、祖辈支持程度(β=0.218)、家庭人均年收入(β=0.173)、治疗依从性(β=0.112)、家庭类型(β=0.105)、患儿年龄(β=-0.100)。结论 早期、正规、坚持治疗的PKU患儿智力发育较好,祖辈支持程度、家庭人家年收入、治疗依从性等因素对患儿的智力发育有重要影响。Objective To analyze the possible influencing factors of intelligent development in children with phenylketonuria(PKU),so as to offer support for formulating related public policies, carrying out health education and family guidance for PKU.Methods A total of 277 children with PKU who were screened and treated in the neonatal disease screening center of Shanxi Maternal and Child Health Hospital from June 2004 to January 2020 were selected as the study subjects.By consulting medical records and telephone follow-up, the general information, result of the latest intelligence test, treatment compliance, and blood phenylalanine(Phe) concentration control condition were collected to analyze the influencing factors of intelligence in children with PKU.Results A total of 277 children with PKU were included.The average intelligence of 277 children with PKU was(94.10±13.57) points, among which 243 cases had the medium or above intellectual level with the proportion of 87.73%,children with critical low intellectual level and developmental disorders accounted for 12.27%,and children with good and excellent intellectual level accounted for 9.74%.The intellectual levels of children who were <3 years old(F=8.108),with caregivers aged 25-35 years old(F=7.224),and caregivers with college education or above(F=44.339) were relatively higher(P<0.05).The intellectual level of urban children was higher than that of rural children(t=-3.336),and the intellectual level of children with higher grandparental support was higher than that of children with lower support(t=-5.549).The intellectual levels of children who started treatment when they were ≤1 month old(t=4.356),whose Phe concentration at diagnosis was <20mg/dL(t=2.148),with good treatment compliance(t=-4.130),and whose Phe concentration was well controlled(t=-3.335) were higher(P<0.05).Multiple linear regression analysis showed that children′s age(β=-0.100) and starting age for treatment(β=-0.581) correlated with the average intellectual level of children with PKU negativel

关 键 词：苯丙酮尿症 苯丙氨酸 智力 影响因素 

分 类 号：R174[医药卫生—妇幼卫生保健]