P341 The association of pulmonary complications and disorders of physical development in patients with cystic fibrosis

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Abstract

Background

Although malnutrition is not a mandatory sign for cystic fibrosis (CF), it presents a common complication in these patients. Several factors idetified in the genesis of poor nutritional status in CF are, such as pancreatic exocrine insufficiency, lack of substitution therapy with pancreatic enzymes, increased energy expenditure due to respiratory complications and other factors.

Background

The aim of present paper was to identify the impact of lung complication, such as chronic Ps.aeruginosa infection and bronchiectsis, on the nutritional status in patients with CF from Republic of Moldova.

Methods

Sixty patients with CF were divided into four age groups: children up to 2 years, 2–5 years, 5–10 years, and 10–18 years. Bacteriological examination of sputum identified the presence of chronic pulmonary infection with Ps.aeruginosa in 51.60% children from our research. Computed tomography of the lungs confirmed the presence of bronchiectasis in 51,33% children with CF whom were radiologically suspected with bronchial dilatation.

Results

Respiratory complications in small children with CF were rare and presented only 8.33% of chronic infection with Ps.aeruginosa and no bronchial deformation on chest X-ray and chest CT. Older children of 2–5 years had a higher rate of pulmonary infection whith Ps.aeruginosa (25.0%) and of bronchiectasis (37.5%). About half of children from th age group 5–10 years showed advanced lung disease: 40.0% – chronic infection with Ps.aeruginosa and 66.7% – bronchiectasis. These complications were very common in patients older than 10 years (Ps.aeruginosa infection – 61.9%, bronchiectasis – 73.2%).

Results

Advanced deformation of the airways, chronic lung infection with aggressive germs trigger the inflammatory processes of the bronchial mucosa, progressively increased with the age (r2=0.92) and had a negative impact on physical development of patients with CF. This vicious circle has been demonstrated in CF patients from our study. In this way, malnutrition showed a high correlation index, both with chronic pulmonary infection with Ps.aeruginosa (r2=0.99) and with bronchiectasis (r2=0.92).

Conclusion

Increased frequency of malnutrition with the age of patients with CF who are receiving good pancreatic enzyme replacement therapy is explained by the association of severe lung complications such as chronic infection with Ps.aeruginosa and bronchiectasis.

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