Magdalena Durda-Masny, Joanna Goździk-Spychalska, Aleksandra John, Wojciech Czaiński, Weronika Stróżewska, Natalia Pawłowska, Jolanta Wlizło, Halina Batura-Gabryel, Anita Szwed
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The study aimed to evaluate the relationship between the severity of mutation, nutritional status, lung function, and Pseudomonas aeruginosa prevalence and survival rate in adult patients with cystic fibrosis.</p><p><strong>Methods: </strong>A study of 124 (68 ♀ and 56 ♂) adults with CF aged 18-51 years were evaluated for (a) type of mutation in the CFTR gene, (b) nutritional status (BMI), (c) lung function (FEV<sub>1</sub>%), and (d) Pseudomonas aeruginosa prevalence. For statistical calculations, Kaplan-Meier analysis of survival, chi-squared test for multiple samples, and logistic regression were used.</p><p><strong>Results: </strong>The type of mutation (χ<sup>2</sup> = 12.73, df = 3, p = 0.005), FEV<sub>1</sub>% (χ<sup>2</sup> = 15.20, df = 2, p = 0.0005), Pseudomonas aeruginosa prevalence (χ<sup>2</sup> = 11.48, df = 3, p = 0.009), and BMI (χ<sup>2</sup> = 31.08, df = 4, p < 0.000) significantly differentiated the probability of survival of patients with CF. 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引用次数: 15
摘要
背景:囊性纤维化(CF)是常见的常染色体隐性遗传病之一。CFTR基因突变类型、营养状况不佳、肺功能衰竭、铜绿假单胞菌感染发展是影响CF患者病情加重和生存率的因素。该研究旨在评估成人囊性纤维化患者突变严重程度、营养状况、肺功能与铜绿假单胞菌患病率和生存率之间的关系。方法:对124例18-51岁成年CF患者(68♀和56♂)进行(A) CFTR基因突变类型、(b)营养状况(BMI)、(c)肺功能(FEV1%)和(d)铜绿假单胞菌患病率的评估。统计计算采用Kaplan-Meier生存分析，多样本卡方检验，logistic回归。结果:突变的类型(χ2 = 12.73,df = 3, p = 0.005), FEV1%(χ2 = 15.20,df = 2, p = 0.0005),铜绿假单胞菌流行(χ2 = 11.48,df = 3, p = 0.009),和BMI(χ2 = 31.08,df = 4, p < 0.000)显著分化的概率CF患者的生存。预期寿命最短的观察患者的一种严重的等位基因突变,FEV1% < 40岁的受试者在他假单胞菌广泛耐药或pandrug-resistant文化,BMI低于18.5 kg/m2的患者。30 ~ 40岁是CF患者寿命最关键的时期。随着铜绿假单胞菌的流行，成年CF患者死亡的风险增加了一倍(OR = 2.06, 95% CI 1.29;2.28)，获得抗生素耐药性时为8倍(OR = 8.11, 95% CI 1.67;38.15)。结论:本研究纳入的所有因素均与囊性纤维化患者的生存率显著相关。
The determinants of survival among adults with cystic fibrosis-a cohort study.
Background: Cystic fibrosis (CF) is one of the most common autosomal recessive diseases. Factors contributing to disease exacerbations and survival rate of CF patients are type of mutation in the CFTR gene, poor nutritional status, lung failure, and infection development by Pseudomonas aeruginosa. The study aimed to evaluate the relationship between the severity of mutation, nutritional status, lung function, and Pseudomonas aeruginosa prevalence and survival rate in adult patients with cystic fibrosis.
Methods: A study of 124 (68 ♀ and 56 ♂) adults with CF aged 18-51 years were evaluated for (a) type of mutation in the CFTR gene, (b) nutritional status (BMI), (c) lung function (FEV1%), and (d) Pseudomonas aeruginosa prevalence. For statistical calculations, Kaplan-Meier analysis of survival, chi-squared test for multiple samples, and logistic regression were used.
Results: The type of mutation (χ2 = 12.73, df = 3, p = 0.005), FEV1% (χ2 = 15.20, df = 2, p = 0.0005), Pseudomonas aeruginosa prevalence (χ2 = 11.48, df = 3, p = 0.009), and BMI (χ2 = 31.08, df = 4, p < 0.000) significantly differentiated the probability of survival of patients with CF. The shortest life expectancy was observed in patients with a severe type of mutation on both alleles, FEV1% < 40, subjects in whom Pseudomonas culture was extensively drug-resistant or pandrug-resistant, and patients whose BMI was lower than 18.5 kg/m2. The period from 30 to 40 years of age was the most critical in CF adults' lifespan. The risk of adults with CF death doubled with Pseudomonas aeruginosa prevalence (OR = 2.06, 95% CI 1.29; 2.28) and eightfold when the bacteria acquired antibiotic resistance (OR = 8.11, 95% CI 1.67; 38.15).
Conclusions: All factors included in the study were significantly related to the survival rate of patients with cystic fibrosis.
期刊介绍：
Journal of Physiological Anthropology (JPA) is an open access, peer-reviewed journal that publishes research on the physiological functions of modern mankind, with an emphasis on the physical and bio-cultural effects on human adaptability to the current environment.
The objective of JPA is to evaluate physiological adaptations to modern living environments, and to publish research from different scientific fields concerned with environmental impact on human life.
Topic areas include, but are not limited to:
environmental physiology
bio-cultural environment
living environment
epigenetic adaptation
development and growth
age and sex differences
nutrition and morphology
physical fitness and health
Journal of Physiological Anthropology is the official journal of the Japan Society of Physiological Anthropology.
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