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Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project

机译：营养状况与囊性纤维化中肺功能之间的关系：德国CF质量保证（CFQA）项目的横截面和纵向分析

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>Background: The German cystic fibrosis (CF) quality assurance (CFQA) project is a patient registry for CF which was founded in 1995. Relevant clinical and laboratory data, respiratory function test results, complications, and CF treatments are entered into the database once a year for each patient. Using the database, a study was undertaken to elucidate the relationship between nutrition and lung function in a large patient cohort by cross sectional and longitudinal analysis. >Methods: A cohort of 3298 patients above 2 years of age was analysed. Patients were grouped according to the presence or absence of malnutrition (wasting and/or stunting). Cross sectional and longitudinal analyses over 2 and 3 years including mixed model analyses were performed. >Results: The prevalence of abnormal weight for height (<90% predicted) increased with age from 19% in children aged <6 years to 38% in adults with CF. Patients with malnutrition had significantly lower mean values of vital capacity, arterial oxygen tension (PO2), and forced expiratory volume in 1 second (FEV1) and higher serum IgG (p<0.05). Pseudomonas aeruginosa infection was also associated with decreased pulmonary function. Malnourished adolescents aged 12–18 years experienced a serious decline in FEV1 of about 20% predicted, whereas mean FEV1 values remained stable at above 80% predicted in adolescents of normal weight. Longitudinal follow up showed that malnourished patients of all ages and those with P aeruginosa infection had significantly worse lung function than their normally nourished counterparts and a greater yearly loss of FEV1 % predicted. During 1 year of observation adolescents who experienced a >5% predicted decrease in weight for height had a concomitant mean loss of FEV1 of 16.5% predicted during that year, whereas patients who gained relative weight had a parallel increase in FEV1 of 2.1% predicted. >Conclusions: These data emphasise the close relationship between nutrition, lung function, and clinical course in CF. Normal body weight and absence of P aeruginosa infection was associated with better preservation of lung function.
机译：>背景：德国囊性纤维化（CF）质量保证（CFQA）项目是CF的患者注册中心，成立于1995年。相关的临床和实验室数据，呼吸功能检查结果，并发症和CF治疗每个患者每年都要输入一次数据库。使用该数据库，进行了一项研究，以通过横断面和纵向分析来阐明大型患者队列中营养与肺功能之间的关系。 >方法：分析了3298名2岁以上的患者。根据是否存在营养不良（浪费和/或发育迟缓）将患者分组。进行了2年和3年的横截面和纵向分析，包括混合模型分析。 >结果：随着年龄的增长，身高异常体重的患病率（预测为<90％）从6岁以下儿童的19％上升为CF成人的38％。营养不良患者的平均肺活量，动脉血氧分压（PO2）和1秒内的呼气量（FEV1）均显着较低，血清IgG较高（p <0.05）。铜绿假单胞菌感染也与肺功能下降有关。营养不良的12-18岁青少年的FEV1严重下降，约为预计的20％，而正常体重的青少年的FEV1平均值保持稳定在80％以上的预测值。纵向随访显示，所有年龄段的营养不良患者和铜绿假单胞菌感染患者的肺功能均比正常营养的患者差得多，预计每年的FEV1％损失更大。在观察的1年中，预计身高体重减轻> 5％的青少年在这一年中伴随的FEV1平均损失为预测的16.5％，而相对体重的患者FEV1的平均升高为预测值的2.1％。 >结论：这些数据强调了CF中营养，肺功能和临床病程之间的密切关系。正常体重和不存在铜绿假单胞菌感染与更好地保留肺功能有关。

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