Diretrizes brasileiras de nutrição em fibrose cística - 2026

Introduction: cystic fibrosis is a multisystem genetic disorder whose clinical course is strongly associated with nutritional status. Advances in diagnosis and treatment, particularly the introduction of CFTR modulators, have substantially changed the nutritional profile of affected individuals, making an update of national nutritional care recommendations necessary. Objective: to update the Brazilian Guidelines for Nutrition in Cystic Fibrosis by establishing a national, evidence-based consensus to guide nutritional assessment, monitoring, and interventions throughout the life course. Methods: these guidelines were developed by a multidisciplinary committee of Brazilian experts based on a review of national and international literature published up to September 2025, followed by a structured consensus process using the Delphi technique. The recommendations addressed nutritional assessment, macronutrient and micronutrient intake, supplementation, oral, enteral, and parenteral nutritional support, pancreatic enzyme replacement therapy, and the nutritional management of comorbidities and special clinical situations. Results: standardized parameters for anthropometric and body composition assessment were established, with emphasis on fat-free mass as a relevant prognostic marker. Updated recommendations for energy and nutrient requirements across different age groups are presented, including specific guidance for individuals receiving CFTR modulators. Clinical criteria for nutritional supplementation and specialized nutritional support were defined, along with monitoring protocols and practical guidance for the nutritional management of comorbidities associated with cystic fibrosis. All recommendations achieved a high level of agreement among the participating experts. Conclusion: this update consolidates a multidisciplinary, evidence-based national consensus, aligned with international guidelines and adapted to the Brazilian context, providing support for clinical decision-making and contributing to the improvement of nutritional care for individuals with cystic fibrosis.