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Abstract：
BACKGROUND: This study aimed to assess medication adherence and demographic, clinical, and psychopathological parameters such as quality of life, depression, and anxiety levels that can affect pediatrics with Wilson\'s Disease (WD).
METHODS: A prospective cohort study was conducted at an outpatient clinic in Turkey among pediatric patients (2 to 18 years) with WD between November 2022 and April 2023. The Medication Adherence Report Scale (MARS-5) as a subjective and Medication Possession Ratio (MPR) as an objective assessment were scored. Physical, genetic and biochemical parameters, the Pediatric Quality of Life Inventory (PedsQL) for both parents and patients, Childhood Depression Inventory, State Trait Anxiety Inventory were also administered.
RESULTS: A total of 30 pediatric outpatients who were prescribed D-penicillamine (n = 27) or trientine (n = 3) as chelators and zinc (n = 29) and pyridoxine (n = 19) as supplements were included. Proteinuria (n = 3), skin rash (n = 2), and gastrointestinal upset (n = 2) were observed. When the correlation between MARS-5 and duration of follow-up was examined, a significant negative correlation was found (p = 0.014). According to MPRs, non-adherence rates (missed doses ≥ 20%) were 29.6%, 17.2% and 5.3% for D-penicillamine, zinc and pyridoxine, respectively. PedsQL scores were higher than those of parents, with a positive correlation between them (p < 0.001). Also, there was a significant positive correlation between PedsQL and State Anxiety Inventory (p < 0.001). Comparing the change in urinary copper levels between different levels of treatment knowledge, significant differences were observed between high- and low levels (p = 0.043).
CONCLUSIONS: Overall, nonadherence rates were 23.3% based on MARS-5 and 5.3-29.6% based on MPR. It is essential to consider factors such as the duration of follow-up, biochemical parameters, treatment knowledge, quality of life and anxiety as potential influencers of medication adherence.
METHODS: A prospective cohort study was conducted at an outpatient clinic in Turkey among pediatric patients (2 to 18 years) with WD between November 2022 and April 2023. The Medication Adherence Report Scale (MARS-5) as a subjective and Medication Possession Ratio (MPR) as an objective assessment were scored. Physical, genetic and biochemical parameters, the Pediatric Quality of Life Inventory (PedsQL) for both parents and patients, Childhood Depression Inventory, State Trait Anxiety Inventory were also administered.
RESULTS: A total of 30 pediatric outpatients who were prescribed D-penicillamine (n = 27) or trientine (n = 3) as chelators and zinc (n = 29) and pyridoxine (n = 19) as supplements were included. Proteinuria (n = 3), skin rash (n = 2), and gastrointestinal upset (n = 2) were observed. When the correlation between MARS-5 and duration of follow-up was examined, a significant negative correlation was found (p = 0.014). According to MPRs, non-adherence rates (missed doses ≥ 20%) were 29.6%, 17.2% and 5.3% for D-penicillamine, zinc and pyridoxine, respectively. PedsQL scores were higher than those of parents, with a positive correlation between them (p < 0.001). Also, there was a significant positive correlation between PedsQL and State Anxiety Inventory (p < 0.001). Comparing the change in urinary copper levels between different levels of treatment knowledge, significant differences were observed between high- and low levels (p = 0.043).
CONCLUSIONS: Overall, nonadherence rates were 23.3% based on MARS-5 and 5.3-29.6% based on MPR. It is essential to consider factors such as the duration of follow-up, biochemical parameters, treatment knowledge, quality of life and anxiety as potential influencers of medication adherence.
摘要：
背景：这项研究旨在评估药物依从性和人口统计学，临床,和精神病理学参数，如生活质量，抑郁症,和焦虑水平会影响威尔逊病（WD）的儿科。
方法：在2022年11月至2023年4月期间，在土耳其的一家门诊诊所对WD儿科患者（2至18岁）进行了一项前瞻性队列研究。对作为主观评价的药物依从性报告量表(MARS-5)和作为客观评价的药物持有比率(MPR)进行评分。Physical,遗传和生化参数，父母和患者的儿科生活质量量表(PedsQL)，儿童抑郁量表,还进行了状态特质焦虑量表。
结果：共包括30名儿科门诊患者，他们被处方为D-青霉胺（n=27）或曲恩汀（n=3）作为螯合剂，锌（n=29）和吡哆醇（n=19）作为补充剂。蛋白尿（n=3），皮疹（n=2），观察到胃肠道不适（n=2）。当检查MARS-5和随访持续时间之间的相关性时，发现显著负相关(p=0.014)。根据MPR，不依从率（错过剂量≥20%）为29.6%，D-青霉胺的17.2%和5.3%，锌和吡哆醇，分别。PedsQL得分高于父母，它们之间呈正相关(p<0.001)。此外，PedsQL与状态焦虑量表呈显著正相关(p<0.001)。比较不同治疗知识水平间尿铜水平的变化,在高水平和低水平之间观察到显著差异(p=0.043).
结论：总体而言，MARS-5的不依从率为23.3%,MPR的不依从率为5.3-29.6%.必须考虑后续行动的持续时间等因素，生化参数，治疗知识,生活质量和焦虑是药物依从性的潜在影响因素。
方法：在2022年11月至2023年4月期间，在土耳其的一家门诊诊所对WD儿科患者（2至18岁）进行了一项前瞻性队列研究。对作为主观评价的药物依从性报告量表(MARS-5)和作为客观评价的药物持有比率(MPR)进行评分。Physical,遗传和生化参数，父母和患者的儿科生活质量量表(PedsQL)，儿童抑郁量表,还进行了状态特质焦虑量表。
结果：共包括30名儿科门诊患者，他们被处方为D-青霉胺（n=27）或曲恩汀（n=3）作为螯合剂，锌（n=29）和吡哆醇（n=19）作为补充剂。蛋白尿（n=3），皮疹（n=2），观察到胃肠道不适（n=2）。当检查MARS-5和随访持续时间之间的相关性时，发现显著负相关(p=0.014)。根据MPR，不依从率（错过剂量≥20%）为29.6%，D-青霉胺的17.2%和5.3%，锌和吡哆醇，分别。PedsQL得分高于父母，它们之间呈正相关(p<0.001)。此外，PedsQL与状态焦虑量表呈显著正相关(p<0.001)。比较不同治疗知识水平间尿铜水平的变化,在高水平和低水平之间观察到显著差异(p=0.043).
结论：总体而言，MARS-5的不依从率为23.3%,MPR的不依从率为5.3-29.6%.必须考虑后续行动的持续时间等因素，生化参数，治疗知识,生活质量和焦虑是药物依从性的潜在影响因素。
