Patient care in cystic fibrosis centers: a real-world analysis in Brazil.

Objective: To analyze the characteristics of cystic fibrosis (CF) care centers (CFCCs) in Brazil.

Methods: A questionnaire was sent to the coordinators of all 51 registered CFCCs between May and September of 2021.

Results: The response rate was 100%.

Chloride Conductance, Nasal Potential Difference and Cystic Fibrosis Pathophysiology.

Purpose: Cystic fibrosis (CF) is a multisystem genetic disease caused by dysfunction of the epithelial anionic channel Cystic Fibrosis Transmembrane conductance Regulator (CFTR). Decreased mucociliary clearance because of thickened mucus is part of the pulmonary disease pathophysiology. It is controversial if the thickened airway surface liquid (ASL) is caused by the deficient chloride secretion and excessive sodium (through ENaC) and water hyperabsorption from the periciliar fluid or by the lack of bicarbonate secretion with relative acidification of the ASL.

Brazilian guidelines for the diagnosis and treatment of cystic fibrosis.

Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life.

Validation of a Nutrition Screening Tool for Pediatric Patients with Cystic Fibrosis.

Background: In cystic fibrosis (CF), nutrition diagnosis is of critical relevance because the early identification of nutrition-related compromise enables early, adequate intervention and, consequently, influences patient prognosis. Up to now, there has not been a validated nutrition screening tool that takes into consideration clinical variables.

Objective: To validate a specific nutritional risk screening tool for patients with CF based on clinical variables, anthropometric parameters, and dietary intake.