exacerbation

恶化
  • 文章类型: Journal Article
    背景:先前的研究一致报道,在2019年冠状病毒病(COVID-19)大流行期间,呼吸道疾病的住院人数减少。然而,大流行对特发性肺纤维化(IPF)入院的影响尚不清楚.
    方法:本研究使用韩国国民健康保险服务数据库中的数据。IPF是根据国际疾病分类第10版(ICD-10)和罕见的顽固性疾病(RID)代码定义的。IPF入院率是通过将IPF入院人数除以IPF患病率来计算的。将COVID-19大流行期间(2020-2021年)的IPF入院率与流行病前期(2017-2019年)的平均入院率进行比较,并表示为比率(RR)。对IPF入院期间接受全身性皮质类固醇治疗的患者进行敏感性分析。
    结果:在根据ICD-10(分析1)定义的IPF患者中,从2020年3月到2021年12月,RR显著下降,但2020年6月和9月除外。同样,在根据ICD-10和RID定义的IPF患者中(分析2),从2020年3月到2021年12月,RR显著下降,但2020年6月和9月除外。在分析1的敏感性分析中,RR在2020年显著下降(0.93;95CI:0.88-0.99;P=0.029),而2021年的RR没有显著差异。分析2的敏感性分析中的RRs在2020年和2021年分别降至0.85(0.79-0.92;P<0.001)和0.82(0.76-0.88;P<0.001)。在亚组分析中,2020年和2021年,男女IPF的入学率显著下降,年龄≥60岁的患者,和所有家庭收入群体。
    结论:在COVID-19大流行期间,IPF的入院率显着下降。这一结果表明,针对COVID-19的预防措施可以有效缓解IPF恶化。因此,假设呼吸道病毒感染与IPF恶化之间存在密切关系.
    BACKGROUND: Previous studies have consistently reported a decrease in hospital admissions for respiratory diseases during the coronavirus disease 2019 (COVID-19) pandemic. However, the impact of the pandemic on idiopathic pulmonary fibrosis (IPF) admissions remains unknown.
    METHODS: This study used data from the Korean National Health Insurance Service database. IPF was defined based on the International Classification of Diseases 10th Revision (ICD-10) and rare intractable disease (RID) codes. The rate of IPF admissions was calculated by dividing the number of IPF admissions by the prevalence of IPF. The rate of IPF admissions during the COVID-19 pandemic (2020-2021) was compared with the mean rate of admissions during the prepandemic period (2017-2019) and presented as the rate ratio (RR). A sensitivity analysis was conducted on patients treated with systemic corticosteroids during IPF admission.
    RESULTS: In patients with IPF defined based on the ICD-10 (analysis 1), the RRs significantly decreased from March in 2020 to December 2021, except for June and September in 2020. Similarly, in patients with IPF defined based on the ICD-10 and RID (analysis 2), the RRs significantly decreased from March 2020 to December 2021, except for June and September 2020. In the sensitivity analysis of analysis 1, the RR significantly decreased in 2020 (0.93; 95%CI: 0.88-0.99; P = 0.029), whereas the RR in 2021 was not significantly different. The RRs in the sensitivity analysis of analysis 2 significantly decreased to 0.85 (0.79-0.92; P < 0.001) in 2020 and 0.82 (0.76-0.88; P < 0.001) in 2021. In the subgroup analysis, the rates of IPF admissions significantly decreased in 2020 and 2021 across both sexes, patients aged ≥ 60 years, and all household income groups.
    CONCLUSIONS: The rate of IPF admissions significantly decreased during the COVID-19 pandemic. This result indicates that preventive measures against COVID-19 may effectively mitigate IPF exacerbation. Therefore, it is assumed that there is a close relationship between respiratory viral infections and IPF exacerbations.
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  • 文章类型: Journal Article
    慢性阻塞性肺疾病(COPD)的特征是持续的呼吸道症状和气流受限。COPD急性加重(AECOPD)是呼吸道症状的急性恶化,这需要额外的治疗,并可能导致健康状况恶化,住院风险和死亡率增加。因此,有必要早期认识和诊断COPD的加重。这篇综述介绍了COPD加重的最新定义,目前的临床评估工具,和目前潜在的生物标志物。本综述还包括移动医疗保健在COPD管理中的早期识别和诊断应用。
    Chronic Obstructive Pulmonary Disease (COPD) is characterized by persistent respiratory symptoms and airflow limitation. Acute exacerbation of COPD (AECOPD) is an acute worsening of respiratory symptoms, which needs additional treatment and can result in worsening health status, increasing risks of hospitalization and mortality. Therefore, it is necessary to early recognize and diagnose exacerbations of COPD. This review introduces the updated definition of COPD exacerbations, the current clinical assessment tools, and the current potential biomarkers. The application of mobile health care in COPD management for early identification and diagnosis is also included in this review.
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  • 文章类型: Journal Article
    目的:COPD和支气管扩张是发病的常见原因,特别是在恶化。呼吸道病原体定植可增加恶化的频率和严重程度。然而,在气道定植患者中,细菌和病毒在恶化时的存在尚未得到很好的研究。
    方法:一项为期6个月的队列研究,研究对象为因支气管扩张(n=26)和/或COPD(n=13)引起的慢性支气管炎以及铜绿假单胞菌或流感嗜血杆菌定植的参与者(n=30)。对参与者进行自我管理教育,并每天收集痰液样本。使用可商购的RT-PCR试剂盒检查基线时(在恶化之前或之后至少14天)和每次恶化时的痰样品的一组34种呼吸道病原体,并与使用用于检测细菌的培养方法获得的结果进行比较。
    结果:参与者提供了29个基线样本和71个恶化样本。在17/29基线样本中,RT-PCR分析证实了培养证明的生物,而12个样本显示与培养结果有差异。大多数恶化(57.7%)与获得新的细菌或病毒无关。而19.8%的人出现了新的细菌,15.7%的新病毒和7%的新病毒和细菌。
    结论:在慢性支气管炎和定植的这一队列参与者中,超过一半的急性加重与新生物无关。然而,26.8%的人在痰中发现了新的细菌种类,这与抗生素治疗有关。在三分之一的参与者中,基线RT-PCR和培养结果不一致。
    OBJECTIVE: COPD and bronchiectasis are common causes of morbidity, particularly around exacerbation. Colonisation with respiratory pathogens can increase the frequency and severity of exacerbations. However, bacterial and viral presence at exacerbation in people with airway colonisation has not been well studied.
    METHODS: A 6-month cohort study of participants (n = 30) with chronic bronchitis due to bronchiectasis (n = 26) and/or COPD (n = 13) and colonisation with Pseudomonas aeruginosa or Haemophilus influenzae was proven on two sputum cultures at exacerbation in the previous 12 months. Participants were provided self-management education and collected sputum samples daily. Sputum samples at baseline (at least 14 days before or after an exacerbation) and at each exacerbation were examined for a panel of 34 respiratory pathogens using commercially available RT-PCR kits and compared to results obtained using culture methods for the detection of bacteria.
    RESULTS: Participants provided 29 baseline samples and 71 samples at exacerbation. In 17/29 baseline samples, RT-PCR analysis confirmed the organism demonstrated by culture, while 12 samples showed a discrepancy from culture results. Most exacerbations (57.7%) were not associated with acquiring new bacteria or viruses, while 19.8% showed new bacteria, 15.7% new viruses and 7% both new viruses and bacteria.
    CONCLUSIONS: Over half of exacerbations were not associated with new organisms in this cohort of participants with chronic bronchitis and colonisation. However, 26.8% demonstrated a new bacterial species in sputum, which is relevant for antibiotic therapy. Baseline RT-PCR and culture results were discordant in one-third of participants.
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  • 文章类型: Journal Article
    背景:虽然哮喘加重仍是患者管理的主要挑战,很少有动物模型来探索潜在的机制。这里,我们建立了哮喘动物模型,可用于研究哮喘加重的病理生理机制和治疗策略.
    方法:使雌性BALB/c小鼠致敏并暴露于PBS或翼状尘螨(DerP)提取物11周。哮喘表型通过肺部炎症评估,支气管高反应性和支气管平滑肌重塑。将哮喘和对照小鼠暴露于聚(I:C)一次或三次以模拟病毒诱导的炎症。
    结果:暴露于DerP后14天,与对照组相比,哮喘小鼠表现出炎症消退,持续的支气管高反应性和支气管平滑肌重塑。在这个阶段,当小鼠单次暴露于聚(I:C)时,对照组和哮喘小鼠的特征是中性粒细胞炎症和支气管高反应性显著增加。当小鼠反复暴露于poly(I:C)时,对照小鼠显示中性粒细胞炎症和支气管高反应性显著降低,而哮喘小鼠经历了这些结果的恶化。
    结论:这项观察性研究报告了一种哮喘小鼠模型,该模型在反复暴露于poly(I:C)后可以加重。我们对对照小鼠肺适应的发现也可能为进一步研究哮喘可能受损的适应机制铺平道路,并提出哮喘恶化是否可能是适应性丧失的问题。
    BACKGROUND: While asthma exacerbations remain a major challenge in patient management, few animal models exist to explore the underlying mechanisms. Here, we established an animal model of asthma that can be used to study pathophysiological mechanisms and therapeutic strategies on asthma exacerbation.
    METHODS: Female BALB/c mice were sensitized and exposed to PBS or Dermatophagoides pteronyssinus (DerP) extract for 11 weeks. Asthmatic phenotype was assessed through lung inflammation, bronchial hyperresponsiveness and bronchial smooth muscle remodeling. Asthmatic and control mice were exposed once or three times to poly(I:C) to simulate virus-induced inflammation.
    RESULTS: Fourteen days after exposure to DerP, asthmatic mice showed resolution of inflammation with sustained bronchial hyperresponsiveness and bronchial smooth muscle remodeling compared to control. At this stage, when mice were subjected to a single exposure to poly(I:C), control and asthmatic mice were characterized by a significant increase in neutrophilic inflammation and bronchial hyperresponsiveness. When mice were repeatedly exposed to poly(I:C), control mice showed a significant decrease in neutrophilic inflammation and bronchial hyperresponsiveness, while asthmatic mice experienced worsening of these outcomes.
    CONCLUSIONS: This observational study report an asthmatic mouse model that can undergo exacerbation after repeated exposure to poly(I:C). Our findings on pulmonary adaptation in control mice may also pave the way for further research into the mechanism of adaptation that may be impaired in asthma and raise the question of whether asthma exacerbation may be a loss of adaptation.
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  • 文章类型: Journal Article
    目的:分析成人哮喘急性发作患者的特点,治疗依从性,和诊所的后续行动。
    方法:纳入2021年5月至2023年6月主要诊断为哮喘加重的≥18岁患者。二次诊断为哮喘加重的患者和未确诊的患者被排除在外。
    结果:共分析了186例患者,63%是女性,平均年龄为49±34岁,平均体重指数(BMI)为26.4±5kg/m2,平均免疫球蛋白E水平为132±235IU/mL(范围:25-2041),平均嗜酸性粒细胞计数为180±443,住院时间为8.6±5天。将一次入院的患者与多次入院的患者进行比较,观察到年龄差异(39±15vs.58±20,p<0.0001),BMI(25.2±3vs.27.4±4,p<0.0003),合并症(15%与60%,p<0.0001),和停留时间(4.5±2vs.11±3,p<0.0001)。在患者中,15%的人患有未确诊的哮喘,28%的人已知哮喘没有维持治疗,23%由初级保健管理,34%其次是肺炎。坚持吸入器的平均测试(TAI)评分为42.5±8分,70%的人表现出反复无常的不坚持,46%的人表现出故意不坚持,21%的人表现出无意识的不依从。
    结论:由于肺科诊所的随访不佳,年轻人群占哮喘急性发作患者的很大比例。治疗优化不足,低坚持。这项研究补充说,有必要改善初级保健中的哮喘治疗方法,以优化治疗,减少诊断不足,避免入院。
    OBJECTIVE: To analyze the characteristics of adult patients admitted for asthma exacerbation and determine optimization, treatment adherence, and follow-up in clinics.
    METHODS: Patients ≥ 18 years old admitted from May 2021 to June 2023 with a primary diagnosis of asthma exacerbation were included. Patients with a secondary diagnosis of asthma exacerbation and those without a confirmed diagnosis were excluded.
    RESULTS: A total of 186 patients were analyzed, 63% were female, with a mean age of 49 ± 34 years, mean body mass index (BMI) of 26.4 ± 5 kg/m2, mean immunoglobulin E level of 132 ± 235 IU/mL (range: 25-2041), mean eosinophils count of 180 ± 443, and length of stay of 8.6 ± 5 days. Comparing patients with one admission to those with multiple admissions, differences were observed in age (39 ± 15 vs. 58 ± 20, p < 0.0001), BMI (25.2 ± 3 vs. 27.4 ± 4, p < 0.0003), comorbidity (15% vs. 60%, p < 0.0001), and length of stay (4.5 ± 2 vs. 11 ± 3, p < 0.0001). Of the patients, 15% had undiagnosed asthma, 28% had known asthma without maintenance therapy, 23% were managed by primary care, and 34% were followed by pneumology. The mean Test of Adherence to Inhalers (TAI) score was 42.5 ± 8 points, with 70% showing erratic non-adherence, 46% showing deliberate non-adherence, and 21% showing unconscious non-adherence.
    CONCLUSIONS: The young population represents a significant percentage of admissions for asthma exacerbation due to poor follow-up in pulmonology clinics, inadequate treatment optimization, and low adherence. This study adds that it is necessary to improve the approach to asthma in primary care to optimize treatment, reduce under-diagnosis, and avoid hospital admissions.
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  • 文章类型: Journal Article
    背景:已知慢性阻塞性肺疾病(COPD)的严重加重会增加心血管事件的风险。然而,这种关联尚未在日本的COPD患者中进行专门调查,其特征可能与西方患者的特征不同(即,西欧,美国,和加拿大)。
    方法:这项纵向回顾性队列研究分析了二级索赔数据,纳入年龄≥40岁的COPD患者(国际疾病分类-10代码J41-J44)。测量随访期间发生的所有恶化。时间依赖性Cox模型用于估计COPD加重后时间段之间关联的风险比(HRs)(与首次恶化之前的时间)和首次住院的严重致死性或非致死性心血管事件的发生。
    结果:分析包括152,712名COPD患者,平均年龄为73.8岁,其中37.6%为女性。在37个月的中位随访中,63,182例(41.4%)患者经历了≥1次加重,13,314例(8.7%)患者经历了≥1次严重心血管事件。COPD加重后,严重心血管事件的风险在前30天增加[校正后HR(aHR)1.44,95%置信区间(CI)1.33~1.55],并且在加重后365天保持升高(aHR1.13,95%CI1.04~1.23).具体来说,急性冠状动脉综合征或心律失常的风险在长达180天的时间内仍然显著增加,以及1年失代偿性心力衰竭的风险。
    结论:在日本COPD患者中,COPD加重后发生严重心血管事件的风险增加,并持续365天,强调需要防止恶化。
    BACKGROUND: Severe exacerbations of chronic obstructive pulmonary disease (COPD) are known to increase the risk of cardiovascular events. However, this association has not been investigated specifically in patients with COPD in Japan, whose characteristics may differ from those of Western patients (i.e., western Europe, the US, and Canada).
    METHODS: This longitudinal retrospective cohort study analyzed secondary claims data and included patients aged ≥ 40 years with COPD (International Classification of Diseases-10 codes J41-J44). All exacerbations occurring during follow-up were measured. Time-dependent Cox models were used to estimate hazard ratios (HRs) for the association between time periods following an exacerbation of COPD (vs. time prior to a first exacerbation) and occurrence of a first hospitalization for a severe fatal or non-fatal cardiovascular event.
    RESULTS: The analysis included 152,712 patients with COPD with a mean age of 73.8 years and 37.6% of whom were female. During a median follow-up of 37 months, 63,182 (41.4%) patients experienced ≥ 1 exacerbation and 13,314 (8.7%) patients experienced ≥ 1 severe cardiovascular event. Following an exacerbation of COPD, the risk of a severe cardiovascular event was increased in the first 30 days [adjusted HR (aHR) 1.44, 95% confidence interval (CI) 1.33-1.55] and remained elevated for 365 days post-exacerbation (aHR 1.13, 95% CI 1.04-1.23). Specifically, the risks of acute coronary syndrome or arrhythmias remained significantly increased for up to 180 days, and the risk of decompensated heart failure for 1 year.
    CONCLUSIONS: Among Japanese patients with COPD, the risk of experiencing a severe cardiovascular event increased following a COPD exacerbation and remained elevated for 365 days, emphasizing the need to prevent exacerbations.
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  • 文章类型: Journal Article
    尚未彻底研究具有最佳峰值吸气流速(PIFR)的慢性阻塞性肺疾病(COPD)患者的特征。这项研究旨在比较PIFR超理想的COPD患者与PIFR最佳和次优的COPD患者的特征。PIFR使用In-CheckDIALG16进行测量,并归类为次优(PIFR低于患者装置要求的PIFR),最优,和超最优(峰值PIFR≥90L/min)。以PIFR次优的COPD患者为参照组,进行分析以确定PIFR相关因素。根据1s用力呼气量(FEV1)%预测值(%pred)进行亚组分析。在韩国七家三级医院的444名支气管扩张剂后确诊的COPD患者中,98、223和123被归类为次优,最优,和超优PIFR组,分别。超优的PIFR组更年轻,男性比例增加,较高的体重指数,去年合并症数量最少,恶化频率较低,以及最高强制肺活量%pred。前一年频繁加重的调整比值比在超理想的PIFR组中低于次优的PIFR组,并且在FEV1%pred<70%的患者中更为明显。具有超最佳PIFR的COPD患者具有不同于具有次最佳和最佳PIFR的患者的临床特征。具有高吸气流量可能是COPD的有利特征。
    Characteristics of chronic obstructive pulmonary disease (COPD) patients with superoptimal peak inspiratory flow rates (PIFR) has not been thoroughly investigated. This study aimed to compare the characteristics between COPD patients with superoptimal PIFR and those with optimal and sub-optimal PIFR. PIFR was measured using In-Check DIAL G16 and categorized into sub-optimal (PIFR lower than that required by the patient\'s device), optimal, and superoptimal (peak PIFR ≥ 90 L/min). Considering COPD patients with sub-optimal PIFR as the reference group, analyses were performed to identify PIFR-related factors. Subgroup analysis was performed according to the forced expiratory volume in 1 s (FEV1) % of the predicted value (%pred). Among 444 post-bronchodilator-confirmed COPD patients from seven tertiary hospitals in South Korea, 98, 223, and 123 were classified into the sub-optimal, optimal, and superoptimal PIFR groups, respectively. The superoptimal PIFR group were younger, had an increased proportion of males, a higher body mass index, lowest number of comorbidities and less frequent exacerbation in the previous year, as well as the highest forced vital capacity %pred. The adjusted odds ratio for frequent exacerbation in the previous year was lower in the superoptimal PIFR group than in the sub-optimal PIFR group and was more pronounced in patients with an FEV1%pred of < 70%. COPD patients with superoptimal PIFR have clinical characteristics different from those patients with the sub-optimal and optimal PIFR. Having a high inspiratory flow may be a favorable trait in COPD.
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  • 文章类型: Journal Article
    哮喘和慢性阻塞性肺疾病是以气道阻塞和慢性炎症为特征的慢性呼吸系统疾病。加重导致症状恶化和增加气流阻塞在两种气道疾病。它们与局部和全身炎症的增加有关。外泌体是细胞来源的含有蛋白质的膜囊泡,脂质,和反映其细胞起源的核酸。通过这些分子的转移,外泌体充当细胞间通讯的介质。通过将其内容物选择性递送到靶细胞,外泌体已被证明参与免疫和炎症的调节。虽然,外泌体已经在不同的疾病中进行了广泛的研究,目前对它们在哮喘和COPD发病机制中的作用知之甚少,尤其是在恶化中。这篇综述旨在系统地评估外泌体在哮喘和COPD急性加重中的潜在作用。
    Asthma and chronic obstructive pulmonary disease are chronic respiratory disorders characterized by airways obstruction and chronic inflammation. Exacerbations lead to worsening of symptoms and increased airflow obstruction in both airways diseases, and they are associated with increase in local and systemic inflammation. Exosomes are cell-derived membrane vesicles containing proteins, lipids, and nucleic acids that reflect their cellular origin. Through the transfer of these molecules, exosomes act as mediators of intercellular communication. Via selective delivery of their contents to target cells, exosomes have been proved to be involved in regulation of immunity and inflammation. Although, exosomes have been extensively investigated in different diseases, little is currently known about their role in asthma and COPD pathogenesis, and particularly in exacerbations. This review aims to systemically assess the potential role of exosomes in asthma and COPD exacerbations.
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  • 文章类型: Journal Article
    背景:粘膜纤毛清除障碍,如囊性纤维化(CF),原发性纤毛运动障碍(PCD)和不明原因的支气管扩张,以呼吸道症状增加的时期为特征,称为肺加重。这些恶化很难预测,并且与肺功能下降和生活质量下降有关。为了优化治疗和保持肺功能,需要非侵入性且可靠的检测方法。呼吸分析可能是这样一种方法。方法:我们系统回顾了现有的呼吸分析文献,以检测粘液纤毛清除障碍的肺加重。提取的数据包括研究设计,测量技术,恶化的定义,已识别的化合物和诊断准确性。结果:在244篇确定的文章中,18人被纳入审查。所有研究包括患有CF的患者和两名也患有PCD的患者。研究之间的年龄和恶化的定义有所不同。有五个使用气相色谱-质谱法测量呼出气中的挥发性有机化合物(VOC),两个使用电子鼻和11测量的有机化合物在呼出的呼吸冷凝液。大多数研究表明,肺加重与一种或多种化合物之间存在显着相关性,主要是碳氢化合物和细胞因子,但这些结果在其他研究中缺乏验证.结论:通过分析呼出气中的化合物来检测肺加重似乎是可能的,但由于结果的主要差异,因此并不接近临床应用。研究设计和恶化的定义。需要更大的研究,纵向设计,国际公认的恶化定义和独立队列结果的验证。
    Background: Disorders of mucociliary clearance, such as cystic fibrosis (CF), primary ciliary dyskinesia (PCD) and bronchiectasis of unknown origin, are characterised by periods with increased respiratory symptoms, referred to as pulmonary exacerbations. These exacerbations are hard to predict and associated with lung function decline and the loss of quality of life. To optimise treatment and preserve lung function, there is a need for non-invasive and reliable methods of detection. Breath analysis might be such a method. Methods: We systematically reviewed the existing literature on breath analysis to detect pulmonary exacerbations in mucociliary clearance disorders. Extracted data included the study design, technique of measurement, definition of an exacerbation, identified compounds and diagnostic accuracy. Results: Out of 244 identified articles, 18 were included in the review. All studies included patients with CF and two also with PCD. Age and the definition of exacerbation differed between the studies. There were five that measured volatile organic compounds (VOCs) in exhaled breath using gas chromatography with mass spectrometry, two using an electronic nose and eleven measured organic compounds in exhaled breath condensate. Most studies showed a significant correlation between pulmonary exacerbations and one or multiple compounds, mainly hydrocarbons and cytokines, but the validation of these results in other studies was lacking. Conclusions: The detection of pulmonary exacerbations by the analysis of compounds in exhaled breath seems possible but is not near clinical application due to major differences in results, study design and the definition of an exacerbation. There is a need for larger studies, with a longitudinal design, international accepted definition of an exacerbation and validation of the results in independent cohorts.
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  • 文章类型: Journal Article
    囊性纤维化(CF)是一种以长期和麻烦的症状为特征的疾病,影响患者的生活。本研究旨在评估和比较波兰CF患者的健康相关生活质量(HRQoL),并确定影响其的因素。研究组由79名患者(6至42岁)组成,他们填写了一份适合年龄的囊性纤维化问卷。从每个患者的医疗记录中收集医疗数据。HRQoL中位数最高的领域是饮食问题(88.89),消化症状(77.78)和身体功能(75.00)。评价最低的领域是社会功能(61.90)。年龄与八个领域负相关,治疗负担最强(rho=-0.474)。身体功能与所有肺活量测定参数呈正相关,和最强烈的FEV1%(rho=0.588)。治疗负担,身体影像和呼吸道症状与所有肺活量测定参数呈正相关,PEF%除外.目前的恶化降低了几乎所有领域的分数,在MANCOVA模型中,它们是区分患者HRQoL的重要因素。单因素分析显示健康状况(F=8.32,p=0.005)和COVID-19大流行(F=5.89,p=0.018)对社会功能领域的显着影响,以及身体图像上的居住地(F=5.60,p=0.21)。随着年龄的增长和恶化期间HRQoL的下降表明,重要的是要关注患者生活的这些方面,并确保他们从医疗保健提供者那里获得必要的支持。
    Cystic fibrosis (CF) is a disease characterized by long-term and troublesome symptoms that affect the patient\'s life. This study aimed to assess and compare the health-related quality of life (HRQoL) of Polish CF patients and identify factors influencing it. The study group consisted of 79 patients (6 to 42 years old), who filled in an age-appropriate Cystic Fibrosis Questionnaire-Revised. Medical data were collected from each patient\'s medical records. The domains with the highest HRQoL median were eating problems (88.89), digestive symptoms (77.78) and physical functioning (75.00). The lowest-rated domain was social functioning (61.90). Age negatively correlated with eight domains, and most strongly with treatment burden (rho = -0.474). Physical functioning positively correlated with all spirometry parameters, and most strongly with FEV1% (rho = 0.588). Treatment burden, body image and respiratory symptoms were positively correlated with all spirometry parameters except PEF%. Present exacerbations reduced scores in almost all domains, and in the MANCOVA model they were a significant factor differentiating patients\' HRQoL. The univariate analysis of MANCOVA showed the significant effects of both health condition (F = 8.32, p = 0.005) and the COVID-19 pandemic (F = 5.89, p = 0.018) on social functioning domain, and of the place of residence on body image (F = 5.60, p = 0.21). A decreasing HRQoL with increasing age and during exacerbations indicates that it is important to focus on these aspects of patients\' lives and ensure they received the necessary support from their healthcare providers.
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