Chinese Journal of Child Health Care ›› 2024, Vol. 32 ›› Issue (2): 191-195.DOI: 10.11852/zgetbjzz2023-0336

• Clinical Research • Previous Articles     Next Articles

Influencing factors of follow-up compliance of special diet therapy in patients with phenylketonuria in four prefectures of Xinjiang Uygur Autonomous Region

ZHOU Shangqin1,2, CHEN Xi3, XUE Shuyuan4, ZHANG Luhan5, DING Guifeng6   

  1. 1. School of Nursing, Hexi University, Zhangye, Gansu 734000, China;
    2. School of Public Health, Xinjiang Medical University, Urumqi, Xinjiang Uygur Autonomous Region 830000, China;
    3. Department of Child Rehabilitation, Urumqi Maternal and Child Health Care Hospital, Urumqi, Xinjiang Uygur Autonomous Region 830000, China;
    4. Urumqi Maternal and Child Health Care Hospital Neonatal Disease Screening Center, Urumqi, Xinjiang Uygur Autonomous Region 830000, China;
    5. Department of Science and Education, Urumqi Maternal and Child Health Care Hospital, Urumqi, Xinjiang Uygur Autonomous Region 830000, China;
    6. Department of Obstetrics, Urumqi Maternal and Child Health Hospital, Urumqi, Xinjiang Uygur Autonomous Region 830000, China
  • Received:2023-04-06 Online:2024-02-10 Published:2024-02-21
  • Contact: DING Guifeng, E-mail:dingguifeng123@126.com

新疆地区四地州苯丙酮尿症患者特食治疗随访依从性的影响因素

周尚琴1,2, 陈曦3, 薛淑媛4, 张璐涵5 , 丁桂凤6   

  1. 1.河西学院护理学院,甘肃 张掖 734000;
    2.新疆医科大学公共卫生学院,新疆维吾尔自治区 乌鲁木齐 830000;
    3.乌鲁木齐市妇幼保健院儿童康复科,新疆维吾尔自治区 乌鲁木齐 830000;
    4.乌鲁木齐市妇幼保健院新生儿疾病筛查中心,新疆维吾尔自治区 乌鲁木齐 830000;
    5.乌鲁木齐市妇幼保健院科教科,新疆维吾尔自治区 乌鲁木齐 830000;
    6.乌鲁木齐市妇幼保健院产科,新疆维吾尔自治区 乌鲁木齐 830000
  • 通讯作者: 丁桂凤,E-mail:dingguifeng123@126.com
  • 作者简介:周尚琴(1984-),女,甘肃人,讲师,硕士学位,主要研究方向为儿少卫生与妇幼保健学。

Abstract: Objective To investigate the influencing factors behind the follow-up compliance of patients with low/no phenylketonuria (PKU) for special medical use, in order to provide a basis for regulating the follow-up of PKU patients and ensuring the effectiveness of special diet treatment. Methods A survey was conducted on PKU patients treated in Urumqi Maternal and Child Health Hospital for over 1 year, from January 2010 to December 2020. Interviews and questionnaires were conducted with their caregivers to collect and analyze the current status of PKU patients undergoing special diet treatment, and to identify the influencing factors behind their compliance with follow-up treatment. Results Patients who had received neonatal disease screening, neonatal gene diagnosis, and maternal Down's screening during pregnancy had better compliance, with statistically significant differences (χ2=5.753, 10.993, 9.189, P<0.05). PKU children with parents who had a college education or above showed significantly higher adherence to special diet treatment (χ2=8.321, 7.415, P<0.05). PKU children with parents having a fixed occupation also showed higher compliance, with a statistically significant difference (χ2=20.626, 7.895, P<0.05). Patient age, interval of buying special diet, number of blood samples sent and enrollment of normal age, all had a significant impact on the follow-up compliance of PKU patients with special diet (χ2=19.443, 8.090, 69.482, 12.001, P<0.05). Conclusions PKU is a treatable genetic metabolic disease. Strengthening health education, formulating standardized follow-up plans and procedures, and improving follow-up treatment compliance are crucial in enhancing the treatment and follow-up effectiveness of PKU patients.

Key words: phenylketonuria, phenylalanine, special diet treatment, compliance

摘要: 目的 了解新疆地区四地州苯丙酮尿症(PKU)患者低/无苯丙氨酸特殊医学用途配方食品(以下简称特食)治疗随访依从性的影响因素,为规范PKU患者的随访,保证特食治疗效果提供依据。方法 调查2010年1月—2020年12月在乌鲁木齐市妇幼保健院治疗随访≥1年以上的PKU患者,对其照护人进行访谈、问卷调查,收集整理PKU患者特食治疗现状,分析特食治疗随访依从性的影响因素。结果 不同筛查情况,接受过新生儿疾病筛查、新生儿基因确诊、母亲孕期唐氏筛查检查的患者依从性较好,差异有统计学意义(χ2=5.753、10.993、9.189,P<0.05)。不同家庭情况,父母亲文化程度为大专及以上者PKU患者的特食治疗依从性较高,差异有统计学意义(χ2=8.321、7.415,P<0.05);父母亲有固定职业的家庭其依从性较高,差异有统计学意义(χ2=20.626、7.895,P<0.05)。年龄、购买特食的间隔时间、送检血片的次数、正常年龄入学情况(χ2=19.443、8.090、69.482、12.001,P<0.05)对PKU患者特食治疗随访依从性存在影响。结论 PKU是一种可以治疗的遗传代谢性疾病,结合临床实际加强健康宣教,制订规范的随访方案及流程,提升随访治疗依从性,为提高PKU患者治疗随访成效起到重要作用。

关键词: 苯丙酮尿症, 苯丙氨酸, 特食治疗, 随访依从性

CLC Number: