Effect of selected factors associated with the clinical course of the disease on nutritional status in children with cystic fibrosis.

Advances in clinical and experimental medicine : official organ Wroclaw Medical University

PubMedID: 25491693

Umlawska W, Krzyzanowska M, Zielinska A, Sands D. Effect of selected factors associated with the clinical course of the disease on nutritional status in children with cystic fibrosis. Adv Clin Exp Med. 2014;23(5):775-83.
BACKGROUND
Malnutrition and delayed growth are commonly seen in children with cystic fibrosis and are indicators of poor prognosis. Understanding the factors that affect growth and nutritional status may improve care, treatment and longevity.

OBJECTIVES
To determine how nutritional status, as estimated using anthropometric measurements, in children with cystic fibrosis is affected by 1) the type of CFTR mutation, 2) colonization by Pseudomonas aeruginosa, and 3) age at diagnosis. The relationship between nutritional status and pulmonary function was also investigated.

MATERIAL AND METHODS
Anthropometric data on height, weight, circumferences and skinfold thicknesses were extracted from the medical histories of 41 boys and 48 girls diagnosed with cystic fibrosis who were treated at the Institute of Mother and Child in Warsaw, Poland. Muscle and fat tissue area were calculated from these measurements. The values for children with CF were compared to the Polish national growth reference charts. Multivariate linear regression tested the effect of three factors on nutritional status: colonization with P. aeruginosa, age at diagnosis, and the type of CFTR mutation.

RESULTS
All values for children with CF were significantly lower than in the reference population. The results show that, in the children examined, nutritional status is more adversely affected than growth. Of the factors related to the clinical picture of the disease, only the presence of respiratory tract colonization by P. aeruginosa had an effect on physical development in the children examined. Neither the type of mutation present nor age at diagnosis had any significant effect.

CONCLUSIONS
Chronic colonization by P. aeruginosa interfered with growth and markedly worsened nutritional status, and was also associated with reductions in both total and lean body mass. Early nutritional intervention can improve nutritional state and pulmonary function in children with CF.