Abstract: Objective To analysis the family environment of children with hyperphenylalanenimia (HPA) and the psychological condition of their mothers,and seek better treatment options. Methods 39 children with hyperphenylalanenimia detected by neonatal screening in Qingdao were compared to 57 matched children.Family Environment Scale-Chinese Version(FES-CV) and Symptom Check List-90(SCL-90) were used to investigate the related factors of the two groups.Results 1)The conflict scores of FES-CV in HPA children were higher than those in controls while the scores of ethical-religion were lower than those in controls (P<0.05).The children with HPA was significantly higher than the control group in the organization scores (P<0.01).2)In the comparison of SCL-90 of the mothers,the scores of total score,total average marks,positive symptom item number,somatization,interpersonal relationships,depression and psychiatric factor in HPA children were higher than the controls,while the scores of negative symptom item number were lower than the controls (P<0.05).The children with HPA was significantly higher than the control group in compulsive symptoms(P<0.01).3)There was no significant differences in FES-CV and SCL-90 between HPA children with different compliance (P>0.05). Conclusion In the treatment of HPA children,psychological intervention to HPA children and their mothers could help to improve the therapeutic effect of the HPA children.

Key words: hyperphenylalanenimia, family environment, mother, mental health

摘要： 目的 研究高苯丙氨酸血症(hyperphenylalanenimia,HPA)患儿的家庭环境及其母亲的心理状况,以寻求更佳的治疗方案。方法 选取青岛市经新生儿疾病筛查确诊的HPA患儿39例作为病例组,选取年龄、性别与病例组匹配且无躯体及精神疾病的正常儿童57例作为对照组,采用家庭环境量表中文版(FES-CV)及症状自评量表(SCL-90)进行测评及研究。结果 1)HPA患儿家庭的矛盾性高于对照组(P<0.05),道德宗教观低于对照组(P<0.05),组织性明显低于对照组(P<0.01);2)母亲SCL-90的比较中,病例组总分、总均分、阳性项目数、躯体化、人际关系、抑郁、精神病性因子得分均高于对照组,阴性项目数得分低于对照组,差异均有统计学意义(P<0.05),强迫症状得分高于对照组,差异有高度统计学意义(P<0.01);3)依从性不同的两组HPA患儿在家庭环境及SCL-90的比较中,各项统计指标均无统计学意义(P>0.05)。 结论 在HPA患儿的治疗过程中,针对其家庭环境及母亲心理存在的问题进行必要的心理干预,有利于改善HPA患儿的治疗效果。

关键词: 高苯丙氨酸血症, 家庭环境, 母亲, 心理健康