Therapeutic Management of Exocrine Pancreatic Insufficiency in Patients with Cystic Fibrosis: An Integrative Review
DOI:
https://doi.org/10.11606/issn.1679-9836.v104i6e-234037Keywords:
Pancreas, lung, cystic fibrosis, treatmentAbstract
The study analyzed evidence on therapy for exocrine pancreatic insufficiency in patients with cystic fibrosis, based on a search conducted in the PubMed and Virtual Health Library (Biblioteca Virtual de Saúde) databases. Studies published between 2019 and 2024 were included, resulting in a final selection of 48 articles, 24 of which focused on therapeutic interventions. The management of pancreatic insufficiency relies on three pillars: pancreatic enzyme replacement therapy (PERT), nutritional support, and continuous monitoring. PERT is essential for correcting malabsorption, utilizing enzymes derived from pigs, but it faces challenges such as patient adherence and variability in treatment response. The need to take multiple capsules and errors in administration are obstacles that require ongoing follow-up. Nutritional support is crucial, involving high-calorie diets and supplementation with fat-soluble vitamins. Despite the importance of nutrition, patient adherence to interventions remains low. CFTR modulators, such as ivacaftor, have emerged as innovative alternatives, but treatment discontinuation reverses the benefits, highlighting the need for further research. The article underscores the importance of integrated and personalized strategies for managing pancreatic insufficiency in cystic fibrosis.
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Copyright (c) 2025 Helena Meurer Heidemann, Laís Corrêa de Carvalho, Bruna Eduarda Weirich, Yasmin Guimarães Rodrigues
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