Introduction:

Adequate nutritional status is essential in patients with Cystic Fibrosis (CF) as said status affects lung function and mortality.

Objective:

To describe the nutritional status of children and adolescents with CF who present for consultation in the Cystic Fibrosis Division of the National Neonatal Screening Program, run by the Ministry of Public Health and Social Welfare (MSPyBS), from February to November 2016.

Materials and methods:

This was an observational, cross-sectional, descriptive study with an analytical component, based on clinical records review of patients with a confirmed diagnosis of CF. The variables studied were: sociodemographics (sex, age at diagnosis, current age and origin), clinical forms at diagnosis and presence of pancreatic sufficiency, anthropometric variables and nutritional status, and pulmonary function (Forced Expiratory Volume un 1 second: FEV1). Parametric and non-parametric descriptive measures, T Student and Pearson correlation were used. Level of significance was defined as p <0.05.

Results:

We analyzed 84 clinical records (56% were female). Average age was 7.5 ± 4.7 years. 56% and came from rural areas of the country. The median age at diagnosis was 4.9 months (64% at ≤ 6 months of age). 41% presented a mixed clinical form (respiratory / digestive) at diagnosis and 22% of diagnosis were due to neonatal detection. The standardized mean values ​​of z Weight/Age, z Size/Age and z Body Mass Index/ Age were -0.6, -1 and - 0.2 respectively. In patients aged <two years (n=9), 11% presented moderate acute malnutrition. In patients ≥ two years of age (n= 75), 60% had adequate nutritional status, 11% were overweight and 3% had obesity. 17.3% of the total showed short stature and 42% fulfilled the nutritional goal in CF. 41% presented chronic colonization by Pseudomonas aeruginosa. The mean pulmonary function (FEV1) was 77.8%, 58.1% presented some degree of pulmonary involvement, with those who were undernourished or at risk for undernourishment showing the most pulmonary compromise (p= 0.0266, Student t test). Children/adolescents who had Pseudomonas aeruginosa had a lower Body Mass Index compared to those who did not (p = 0.0324, Student t test) and those who had a lower BMI had lower pulmonary function (positive Pearson correlation, p = 0.001 ).

Conclusion:

Children under two years of age did not show short stature. In children older than two years, six out of 10 had an adequate nutritional status by Body Mass Index/Age. However, stature was compromised in more than half of this group of children. 14% have malnutrition due to excess. Almost 6 out of 10 CF patients present some degree of pulmonary involvement, with those children at risk of malnutrition or undernourished showing more pulmonary compromise.

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