评估苯丙酮尿症患者及其父母的压力水平和生活困难。在2020年1月至2020年6月之间,包括156名接受定期检查的PKU患者及其父母。父母被要求完成育儿压力指数,Zarit负担量表,和优势和困难问卷(SDQ),11岁以上的儿童被要求填写罗森博格自尊量表,状态特质焦虑量表,和SDQ。我们发现Rosenberg自尊量表与诊断年龄之间存在显着负相关(r=-0.27,P=.035),母亲的年龄(r=-0.33,P=0.009),和父亲的年龄(r=-0.38,P=.004)。状态-特质焦虑量表与患者年龄呈显著正相关(r=0.36,P=.006),母亲的年龄(r=0.29,P=.031)和父亲的年龄(r=0.38,P=.024)。在SDQ的子形式中,诊断时情绪问题与血清苯丙氨酸(Phe)水平呈显著正相关(r=0.35,P=0.036),总分与临床检查时血清Phe水平呈显著正相关(r=-0.34,P=.004),社会问题与父亲年龄呈显著负相关(r=-0.34,P=0.047)。我们发现Zarit负担量表与兄弟姐妹数量之间存在显着正相关(r=0.195,P=0.023)。在SDQ的父形式中,情绪问题与患者年龄呈显著正相关(r=0.217,P=0.032),同伴问题与诊断时的年龄呈显著正相关(r=0.211,P=0.037),行为问题(r=0.203,P=0.045),注意缺陷和多动症(r=0.203,P=0.045)与诊断时的血清Phe水平呈显着正相关。苯丙酮尿症是难以应付的患者和他们的父母,因为饮食义务,饮食所需配方的高支出,定期临床检查的要求,以及精神残疾和精神疾病的可能发展。应对患者及其家人进行心理评估,并在需要时提供支持。
To assess stress levels and life hardships of patients with phenylketonuria and their parents. Between January 2020 and June 2020, 156 patients with PKU and their parents who arrived for regular examinations were included. Parents were asked to complete the parenting stress index, Zarit Burden Scale, and the Strengths and Difficulties Questionnaire (SDQ), and children over the age of 11 were asked to fill the Rosenberg Self-Esteem Scale, the State-Trait Anxiety Inventory, and the SDQ. We found a significant negative correlation between the Rosenberg Self-Esteem Scale and age at diagnosis (r = -0.27, P = .035), mother\'s age (r = -0.33, P = .009), and father\'s age (r = -0.38, P = .004). There was a significant positive correlation between the State-Trait Anxiety Inventory and patient\'s age (r = 0.36, P = .006), mother\'s age (r = 0.29, P = .031) and father\'s age (r = 0.38, P = .024). In the child form of the SDQ, emotional problems were significantly positively correlated with serum
phenylalanine (Phe) levels at diagnosis (r = 0.35, P = .036), total points were significantly positively correlated with serum Phe levels at clinical examination (r = -0.34, P = .004), and social problems were significantly negatively correlated with the father\'s age (r = -0.34, P = .047). We found a significant positive correlation between the Zarit Burden Scale and number of siblings (r = 0.195, P = .023). In the parent form of the SDQ, emotional problems were significantly positively correlated with patient age (r = 0.217, P = .032), peer problems were significantly positively correlated with age at diagnosis (r = 0.211, P = .037), behavioral problems (r = 0.203, P = .045), and attention deficit and hyperactivity (r = 0.203, P = .045) were significantly positively correlated with serum Phe levels at diagnosis. Phenylketonuria is difficult to cope with both for the patients and their parents because of diet obligation, high expenditures for the formulas required for the diet, requirement of regular clinical examinations, and possible development of mental disability and psychiatric disorders. Patients and their families should be psychologically evaluated and support should be provided if needed.