关键词: Cystic fibrosis Pulmonary disease Pulmonary exacerbation Pulmonary exacerbation outcomes Respiratory disease Respiratory symptoms Symptom Symptom clustering Symptom phenotype Symptom phenotyping

来  源:   DOI:10.1016/j.jcf.2024.05.014

Abstract:
BACKGROUND: People with cystic fibrosis (PwCF) experience frequent symptoms associated with chronic lung disease. A complication of CF is a pulmonary exacerbation (PEx), which is often preceded by an increase in symptoms and a decline in lung function. A symptom cluster is when two or more symptoms co-occur and are related; symptom clusters have contributed meaningful knowledge in other diseases. The purpose of this study is to discover symptom clustering patterns in PwCF during a PEx to illuminate symptom phenotypes and assess differences in recovery from PExs.
METHODS: This study was a secondary, longitudinal analysis (N = 72). Participants at least 10 years of age and being treated with intravenous antibiotics for a CF PEx were enrolled in the United States. Symptoms were collected on treatment days 1-21 using the CF Respiratory Symptom Diary (CFRSD)-Chronic Respiratory Symptom Score (CRISS). K-means clustering was computed on day 1 symptom data to detect clustering patterns. Linear regression and multi-level growth models were performed.
RESULTS: Symptoms significantly clustered based on severity: low symptom (LS)-phenotype (n = 42), high symptom (HS)-phenotype (n = 30). HS-phenotype had worse symptoms and CRISS scores (p< 0.01) than LS-phenotype. HS-phenotype was associated with spending 5 more nights in the hospital annually (p< 0.01) than LS-phenotype. HS-phenotype had worse symptoms over 21 days than LS-phenotype (p< 0.0001).
CONCLUSIONS: Symptoms significantly cluster on day 1 of a CF-PEx. PwCF with HS-phenotype spend more nights in the hospital and are less likely to experience the same resolution in symptoms by the end of PEx treatment than LS-phenotype.
摘要:
背景:囊性纤维化(PwCF)患者会出现与慢性肺部疾病相关的频繁症状。CF的并发症是肺加重(PEx),通常在症状增加和肺功能下降之前。症状群是指两个或多个症状共同出现并相关;症状群在其他疾病中贡献了有意义的知识。这项研究的目的是发现PEx期间PwCF中的症状聚类模式,以阐明症状表型并评估从PEx恢复的差异。
方法:这项研究是次要的,纵向分析(N=72)。在美国招募了至少10岁并正在接受CFPEx静脉注射抗生素治疗的参与者。使用CF呼吸症状日记(CFRSD)-慢性呼吸症状评分(CRISS)在治疗第1-21天收集症状。在第1天症状数据上计算K均值聚类以检测聚类模式。进行线性回归和多水平生长模型。
结果:根据严重程度,症状显着聚集:低症状(LS)表型(n=42),高症状(HS)表型(n=30)。HS-表型具有比LS-表型更差的症状和CRISS评分(p<0.01)。与LS-表型相比,HS-表型与每年在医院多住5晚相关(p<0.01)。HS-表型在21天内的症状比LS-表型更差(p<0.0001)。
结论:症状在CF-PEx的第1天显著聚集。与LS-表型相比,具有HS-表型的PwCF在医院花费更多的夜晚,并且在PEx治疗结束时不太可能经历相同的症状消退。
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