Abstract：
UNASSIGNED: Personalized medicine requires the assessment of the impact of health care interventions on Health-Related Quality of Life.
UNASSIGNED: We run an observational study of HRQoL in 140 CVID patients with biannual assessments over 8 years using a disease-specific tool, the CVID_QoL, and the GHQ questionnaires. Factors influencing changes in HRQoL scores were identified using multiple linear regression models with a stepwise procedure.
UNASSIGNED: Infections frequency, female gender, and chronic enteropathy were associated with worse global CVID_QoL scores. The presence of permanent organ damage and older age contributed to the perception of being at risk of health deterioration, while chronic enteropathy was associated with fatigue. The presence of permanent organ damage was also associated with perceived difficulties in usual activities. The frequency of infections was the main risk factor for difficulties in long-term planning and perceptions of vulnerability. Before COVID-19, improved HRQoL scores were associated with reduced respiratory infections and changes in immunoglobulin replacement route and setting. The COVID-19 pandemic caused a sudden deterioration in all HRQoL dimensions, and a further deterioration in the emotional dimension was observed during the pandemic period. Patients who died during the study had worse CVID_QoL scores at all time points, confirming that HRQoL performance is strongly related to patient outcome.
UNASSIGNED: Periodic HRQoL assessments are needed to capture relevant issues that change over time in patients affected by long-term chronic conditions such CVID, possibly identifying areas of intervention.
UNASSIGNED: We run an observational study of HRQoL in 140 CVID patients with biannual assessments over 8 years using a disease-specific tool, the CVID_QoL, and the GHQ questionnaires. Factors influencing changes in HRQoL scores were identified using multiple linear regression models with a stepwise procedure.
UNASSIGNED: Infections frequency, female gender, and chronic enteropathy were associated with worse global CVID_QoL scores. The presence of permanent organ damage and older age contributed to the perception of being at risk of health deterioration, while chronic enteropathy was associated with fatigue. The presence of permanent organ damage was also associated with perceived difficulties in usual activities. The frequency of infections was the main risk factor for difficulties in long-term planning and perceptions of vulnerability. Before COVID-19, improved HRQoL scores were associated with reduced respiratory infections and changes in immunoglobulin replacement route and setting. The COVID-19 pandemic caused a sudden deterioration in all HRQoL dimensions, and a further deterioration in the emotional dimension was observed during the pandemic period. Patients who died during the study had worse CVID_QoL scores at all time points, confirming that HRQoL performance is strongly related to patient outcome.
UNASSIGNED: Periodic HRQoL assessments are needed to capture relevant issues that change over time in patients affected by long-term chronic conditions such CVID, possibly identifying areas of intervention.
摘要：
■个性化医疗需要评估卫生保健干预措施对健康相关生活质量的影响。
■我们在140例CVID患者中进行HRQoL的观察性研究，使用疾病特异性工具进行为期8年的半年评估，CVID_QoL，和GHQ问卷。使用逐步程序的多元线性回归模型确定影响HRQoL评分变化的因素。
■感染频率，女性性别,和慢性肠病与较差的全球CVID_QoL评分相关。永久性器官损伤的存在和年龄的增长导致人们认为健康有恶化的风险，而慢性肠病与疲劳有关。永久性器官损伤的存在也与通常活动中的感知困难有关。感染的频率是长期规划困难和对脆弱性的认识的主要风险因素。在COVID-19之前，HRQoL评分的改善与呼吸道感染的减少以及免疫球蛋白替代途径和设置的变化有关。COVID-19大流行导致所有HRQoL维度的突然恶化，在大流行期间，观察到情绪方面的进一步恶化。在研究期间死亡的患者在所有时间点的CVID_QoL评分均较差，确认HRQoL表现与患者预后密切相关。
■需要定期进行HRQoL评估，以捕获受长期慢性疾病（如CVID）影响的患者随时间变化的相关问题。可能确定干预领域。
■我们在140例CVID患者中进行HRQoL的观察性研究，使用疾病特异性工具进行为期8年的半年评估，CVID_QoL，和GHQ问卷。使用逐步程序的多元线性回归模型确定影响HRQoL评分变化的因素。
■感染频率，女性性别,和慢性肠病与较差的全球CVID_QoL评分相关。永久性器官损伤的存在和年龄的增长导致人们认为健康有恶化的风险，而慢性肠病与疲劳有关。永久性器官损伤的存在也与通常活动中的感知困难有关。感染的频率是长期规划困难和对脆弱性的认识的主要风险因素。在COVID-19之前，HRQoL评分的改善与呼吸道感染的减少以及免疫球蛋白替代途径和设置的变化有关。COVID-19大流行导致所有HRQoL维度的突然恶化，在大流行期间，观察到情绪方面的进一步恶化。在研究期间死亡的患者在所有时间点的CVID_QoL评分均较差，确认HRQoL表现与患者预后密切相关。
■需要定期进行HRQoL评估，以捕获受长期慢性疾病（如CVID）影响的患者随时间变化的相关问题。可能确定干预领域。
