Gozdzik J, Cofta S, Piorunek T, Batura-Gabryel H, Kosicki J
Department of Respiratory Diseases, University of Medical Sciences, Poznan, Poland.
J Physiol Pharmacol. 2008 Dec;59 Suppl 6:253-60.
Cystic fibrosis (CF) is a multisystem autosomal recessive disorder caused by the mutation of a single gene that encodes for the CF transmembrane regulator protein. Clinically, CF is characterized by chronic pulmonary infection, pancreatic insufficiency, and excessive losses of sweat electrolytes. Along with lung function, nutritional status appears one of the most important prognostic indicators in CF patients. In this study we examined the relationship between nutritional status and pulmonary function in adult CF patients. A group of 39 CF patients (mean age 23.9 +/-3.7 years) was studied. The mean value of body mass index (BMI) was 19.5 +/-2.9kg/m(2) (12.8-24.9kg/m(2)). The patients were grouped according to the presence or absence of malnutrition. Malnutrition was established in 11 patients (28.2%), 5 patients suffered from severe malnutrition. 28 patients (71.8%) had a normal nutritional status, but according to ESPEN guidelines, 9 of those patients were at risk of malnutrition. Statistical analysis revealed a significant difference between malnourished and not malnourished patients with respect to FEV(1)% and FVC%. Moreover, the patients with malnutrition were significantly more frequently colonized by P. aeruginosa and fungi and less so by MSSA.
囊性纤维化(CF)是一种多系统常染色体隐性疾病,由单个基因突变引起,该基因编码囊性纤维化跨膜调节蛋白。临床上,CF的特征是慢性肺部感染、胰腺功能不全以及汗液电解质过度流失。除肺功能外,营养状况似乎是CF患者最重要的预后指标之一。在本研究中,我们调查了成年CF患者营养状况与肺功能之间的关系。研究了一组39例CF患者(平均年龄23.9±3.7岁)。体重指数(BMI)的平均值为19.5±2.9kg/m²(12.8 - 24.9kg/m²)。根据是否存在营养不良对患者进行分组。11例患者(28.2%)存在营养不良,5例患者患有严重营养不良。28例患者(71.8%)营养状况正常,但根据欧洲临床营养与代谢学会(ESPEN)指南,其中9例患者存在营养不良风险。统计分析显示,营养不良和非营养不良患者在第1秒用力呼气容积百分比(FEV₁%)和用力肺活量百分比(FVC%)方面存在显著差异。此外,营养不良患者被铜绿假单胞菌和真菌定植的频率明显更高,而被甲氧西林敏感金黄色葡萄球菌定植的频率更低。