Abstract：
BACKGROUND: There are important advances in the management of bacterial infection in patients with cystic fibrosis (CF), but there are many gaps in the field of fungal infections.
OBJECTIVE: The aim of this study was to analyse whether chronic respiratory filamentous fungal colonization had clinical impact and whether antifungal treatment can change the disease.
METHODS: The prospective, bicentric and descriptive study was carried out within a 3-year follow-up period, with four-month periodicity medical controls. Adult patients from two CF units of tertiary hospitals were included. Clinical, microbiological, analytical and spirometric variables were collected. Quality of life was evaluated in a subgroup, using the Spanish version of the Revised Cystic Fibrosis Quality of Life Questionnaire (CFQ-R). To statistically analyze the evolution of forced expiratory along time (volume of air blown out in 1 second -FEV1-) and the forced vital capacity (FVC), mixed linear models were carried out.
RESULTS: From the ninety-eight patients under study, 40 suffered chronic filamentous fungal colonization. The presence of filamentous fungi in airway was associated to an annual fall of FEV1 and FVC of 0.029 and 0.017 litres, respectively (p<0.001). In addition, worse quality of life based on CFQ-R, significant when concerning physical condition and emotional state, was also linked with the fungal colonization. Protocolized antifungal therapy, nebulized or oral, improved FEV1 in 0.023 and 0.024 litres per year, respectively (p<0.001).
CONCLUSIONS: Chronic filamentous fungal colonization in patients with CF is associated with a significant annual decline of lung function that persists over time. Chronic antifungal therapy slows down this progression, mainly in the patient with more advanced disease.
OBJECTIVE: The aim of this study was to analyse whether chronic respiratory filamentous fungal colonization had clinical impact and whether antifungal treatment can change the disease.
METHODS: The prospective, bicentric and descriptive study was carried out within a 3-year follow-up period, with four-month periodicity medical controls. Adult patients from two CF units of tertiary hospitals were included. Clinical, microbiological, analytical and spirometric variables were collected. Quality of life was evaluated in a subgroup, using the Spanish version of the Revised Cystic Fibrosis Quality of Life Questionnaire (CFQ-R). To statistically analyze the evolution of forced expiratory along time (volume of air blown out in 1 second -FEV1-) and the forced vital capacity (FVC), mixed linear models were carried out.
RESULTS: From the ninety-eight patients under study, 40 suffered chronic filamentous fungal colonization. The presence of filamentous fungi in airway was associated to an annual fall of FEV1 and FVC of 0.029 and 0.017 litres, respectively (p<0.001). In addition, worse quality of life based on CFQ-R, significant when concerning physical condition and emotional state, was also linked with the fungal colonization. Protocolized antifungal therapy, nebulized or oral, improved FEV1 in 0.023 and 0.024 litres per year, respectively (p<0.001).
CONCLUSIONS: Chronic filamentous fungal colonization in patients with CF is associated with a significant annual decline of lung function that persists over time. Chronic antifungal therapy slows down this progression, mainly in the patient with more advanced disease.
摘要：
背景：囊性纤维化（CF）患者的细菌感染管理有重要进展，但是真菌感染领域存在许多空白。
目的：本研究的目的是分析慢性呼吸道丝状真菌定植是否具有临床影响以及抗真菌治疗是否可以改变疾病。
方法：前瞻性，在3年的随访期内进行了双中心和描述性研究，有四个月的周期性医疗控制。包括来自三级医院的两个CF单位的成年患者。临床,微生物,收集分析和肺活量测定变量。在一个亚组中评估了生活质量，使用西班牙语版本的修订的囊性纤维化生活质量问卷（CFQ-R）。统计分析用力呼气随时间（1秒内吹出的空气量-FEV1-）和用力肺活量（FVC）的演变。进行了混合线性模型。
结果：从研究的98名患者中，40遭受慢性丝状真菌定植。气道中丝状真菌的存在与FEV1和FVC每年下降0.029和0.017升有关，分别(p<0.001)。此外,基于CFQ-R的生活质量更差，当涉及身体状况和情绪状态时，也与真菌定植有关。促生抗真菌治疗，雾化或口服，每年改进的FEV1为0.023和0.024升，分别(p<0.001)。
结论：CF患者的慢性丝状真菌定植与肺功能每年显著下降相关，并随时间持续。慢性抗真菌治疗减缓了这种进展，主要是晚期疾病的患者。
目的：本研究的目的是分析慢性呼吸道丝状真菌定植是否具有临床影响以及抗真菌治疗是否可以改变疾病。
方法：前瞻性，在3年的随访期内进行了双中心和描述性研究，有四个月的周期性医疗控制。包括来自三级医院的两个CF单位的成年患者。临床,微生物,收集分析和肺活量测定变量。在一个亚组中评估了生活质量，使用西班牙语版本的修订的囊性纤维化生活质量问卷（CFQ-R）。统计分析用力呼气随时间（1秒内吹出的空气量-FEV1-）和用力肺活量（FVC）的演变。进行了混合线性模型。
结果：从研究的98名患者中，40遭受慢性丝状真菌定植。气道中丝状真菌的存在与FEV1和FVC每年下降0.029和0.017升有关，分别(p<0.001)。此外,基于CFQ-R的生活质量更差，当涉及身体状况和情绪状态时，也与真菌定植有关。促生抗真菌治疗，雾化或口服，每年改进的FEV1为0.023和0.024升，分别(p<0.001)。
结论：CF患者的慢性丝状真菌定植与肺功能每年显著下降相关，并随时间持续。慢性抗真菌治疗减缓了这种进展，主要是晚期疾病的患者。
