Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis

Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis
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DOI:
10.1136/thx.2010.139493
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发表时间:
2011-05-01
期刊:
影响因子:
10
通讯作者:
Sly, Peter D.
Sly, Peter D.
中科院分区:
医学1区
文献类型:
--
作者:
Ranganathan, Sarath C.;Parsons, Faith;Sly, Peter D.

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改善营养是新生儿囊性纤维化(CF)筛查计划的主要益处,并与更好的临床结果相关。据推测,CF婴儿早期肺部炎症和感染与营养不良有关。方法对CF患儿在诊断后不久及1、2、3岁时进行体重、身高、肺部炎症及支气管肺泡灌洗(BAL)感染的评估。身体质量指数(BMI)用z分数表示。测定BAL的炎症细胞和细胞因子(白细胞介素1 β (IL-1 β)、IL-6、IL-8和肿瘤坏死因子α (TNF α))、游离中性粒细胞弹性酶活性和髓过氧化物酶。在调整潜在混杂因素后,使用混合效应模型评估肺部炎症、肺部感染(金黄色葡萄球菌和铜绿假单胞菌)和BMI z-score之间的纵向关联。结果本组共42例婴儿,其中男16例(38%);39例(93%)胰腺功能不全;36例通过新生儿筛查诊断(中位年龄4周),6例通过早期临床诊断(胎粪肠梗阻)。31例(74%)接受了抗葡萄球菌抗生素治疗。每次评估时,超过三分之二的患者无症状。平均BMI z评分在诊断时为-1.5,在1年、2年和3年分别为0.5、-0.2和-0.1。中性粒细胞弹性酶和金黄色葡萄球菌感染与较低的BMI相关,而年龄(p=0.01)和抗葡萄球菌抗生素(p=0.013)与BMI升高相关。平均而言,游离中性粒细胞弹性酶活性每增加log(10)与BMI z-score降低0.43 (95% CI 0.06至0.79)相关。CF患者早期营养状况与潜在的肺部病理生理有关,需要更好地了解这些关系。需要研究来评估干预措施是否能减少肺部炎症和改善营养。早期监测将使这种有针对性的干预成为可能,目的是改善这些重要的临床结果。
Introduction Improved nutrition is the major proven benefit of newborn screening programmes for cystic fibrosis (CF) and is associated with better clinical outcomes. It was hypothesised that early pulmonary inflammation and infection in infants with CF is associated with worse nutrition.Methods Weight, height and pulmonary inflammation and infection in bronchoalveolar lavage (BAL) were assessed shortly after diagnosis in infants with CF and again at 1, 2 and 3 years of age. Body mass index (BMI) was expressed as z-scores. Inflammatory cells and cytokines (interleukin 1 beta (IL-1 beta), IL-6, IL-8 and tumour necrosis factor alpha (TNF alpha)), free neutrophil elastase activity and myeloperoxidase were measured in BAL. Mixed effects modelling was used to assess longitudinal associations between pulmonary inflammation, pulmonary infection (Staphylococcus aureus and Pseudomonas aeruginosa) and BMI z-score after adjusting for potential confounding factors.Results Forty-two infants were studied (16 (38%) male; 39 (93%) pancreatic insufficient); 36 were diagnosed by newborn screening (at median age 4 weeks) and six by early clinical diagnosis (meconium ileus). Thirty-one (74%) received antistaphylococcal antibiotics. More than two-thirds were asymptomatic at each assessment. Mean BMI z-scores were -1.5 at diagnosis and 0.5, -0.2 and -0.1 at 1, 2 and 3 years, respectively. Neutrophil elastase and infection with S aureus were associated with lower BMI, whereas age (p=0.01) and antistaphylococcal antibiotics (p=0.013) were associated with increased BMI. On average, each log(10) increase in free neutrophil elastase activity was associated with a 0.43 (95% CI 0.06 to 0.79) reduction in BMI z-score.Discussion Early nutritional status is associated with the underlying pulmonary pathophysiology in CF, and better understanding of these relationships is required. Studies are required to assess whether interventions can decrease pulmonary inflammation and improve nutrition. Early surveillance will enable such targeted interventions with the aim of improving these important clinical outcomes.