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Undernutrition and Clinical Deterioration in Children with Cystic Fibrosis: Findings from a Prospective Study

Bruna B DA Silva1, Aline Daiane Schlindwein2, Rosemeri M DA Silva3

  • 1University of Southern Santa Catarina, Postgraduate Program in Health Sciences, Av. José Acácio Moreira, 787, Dehon, 88704-900 Tubarão, SC, Brazil.

Anais Da Academia Brasileira De Ciencias
|February 19, 2026
PubMed

Insights

Poor nutrition in pediatric Cystic Fibrosis patients is linked to earlier symptom onset and gastroesophageal reflux disease. Maintaining good nutritional status is crucial for managing this genetic disorder.

Area of Science:

  • Pediatric Pulmonology
  • Clinical Nutrition
  • Genetic Disorders

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting epithelial cell function, leading to pulmonary issues and poor weight gain.
  • Nutrition is vital for survival and quality of life in CF patients.
  • Understanding the impact of nutritional status on clinical outcomes is essential for pediatric CF care.

Purpose of the Study:

  • To investigate the association between nutritional status and clinical condition in pediatric Cystic Fibrosis patients.
  • To evaluate the influence of nutritional status on disease progression and related symptoms.

Main Methods:

  • Prospective cohort study involving 102 pediatric CF patients initially, with 88 completing the study.
  • Data collected included sociodemographic, birth, clinical, anthropometric, food intake, Shwachman-Kulczycki scores, and lung function.
  • Statistical analysis involved calculating relative risk (RR) and 95% confidence intervals (CI).

Main Results:

  • Patients with nutritional status below the 50th percentile experienced earlier CF symptoms (0.96 months vs. 5.08 months).
  • Impaired nutritional status was associated with higher rates of gastroesophageal reflux disease (53.2%) and muscle tissue depletion (70%).
  • Lower body weight was observed in patients with suboptimal nutritional status.

Conclusions:

  • Suboptimal nutritional status in pediatric CF patients correlates with earlier symptom onset, gastroesophageal reflux disease, muscle depletion, and lower body weight.
  • Nutritional status significantly impacts the clinical presentation of Cystic Fibrosis in children and adolescents.
  • Further research may explore interventions to improve nutritional status and clinical outcomes in CF.

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