Association of nutritional status and pulmonary function in children with cystic fibrosis

Multiple studies have shown that nutritional status is a strong predictor of morbidity and mortality in patients with cystic fibrosis (CF). Since CF is characterized by progressive lung disease, it could be argued that the underlying lung disease is what determines the nutritional failure seen in most patients. This review will summarize the data available from studies that have attempted to better define this relation and also present a review of the possible mechanisms involved taken from both observational and interventional studies.

Longitudinal studies with sufficiently large follow-up times have demonstrated that young underweight patients have worst pulmonary function outcomes. More importantly, these studies concur in that the yearly change in growth parameters has a significant effect on the rate at which pulmonary function develops. Although the mechanisms behind this important association are yet unclear, there is some suggestion from interventional studies that the accrual of lean body mass is the factor that is involved in the preservation of lung function.

Nutritional status strongly influences pulmonary health among CF patients. Therefore, aggressive nutritional support aiming at achieving normal growth patterns should lead to adequate development of lung function and maintenance of pulmonary health. However, more research is required with long-term longitudinal studies to better identify the most critical nutritional characteristics influencing this process as well as the most effective nutritional interventions.