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Objective: To verify the influence of clinical and socioeconomic factors on the nutritional status of pediatric CF patients treated at a referral care facility in the State of Piauí and to describe their general profile. METHODS: This was an observational cross-sectional retrospective descriptive study with secondary data analysis, performed by data from medical records of 23 patients under 16 years of age with confirmed cystic fibrosis, attended at 2018, were collected. RESULTS: There was a slight predominance of male and brown, homozygous (p=0.05), from southeast Piaui, in school age group. Age at diagnosis was 2.0±3.0 years, with 13% asymptomatic. Respiratory symptoms and growth deficits were the main initial manifestations. 47.8% were diagnosed by neonatal screening. Most had good and excellent score for the Shwachman-Kulczycki score, average of 78.5±16.7. Most were eutrophic, with adequate Z-score values. Influence of nutritional status on the Shwachman-Kulczycki score was noted, and the latter was found to be significantly lower (p=0.037) in patients with nutritional deficit. DISCUSSION: There was a delay in the diagnosis of these patients, with low coverage of neonatal screening tests. The socioeconomic and clinical aspects did not influence the nutritional status, except for the severity score that was worse in those with nutritional deficit. CONCLUSION: Nutritional status influenced the severity score, confirming the importance of adequate management and nutritional monitoring for improving the quality of life.

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