Highlights ■ SARS-CoV-2 infection leads to mild-to-moderate disease in children with cystic fibrosis. ■ No worsening of cystic fibrosis was observed months after infection. ABSTRACT Objective: This study aimed to evaluate the clinical manifestations of SARS-CoV-2 in children and adolescents with cystic fibrosis. Methods: This was a case-control analysis of clinical variables and pulmonary function test results in 43 children with cystic fibrosis, 17 (39.5%) of whom tested positive for SARS-CoV-2. Results: The infected children exhibited a higher frequency of […]

ABSTRACT Objective To develop a scientific consensus on nutrition in cystic fibrosis. Methods Sixteen coordinators elaborated relevant questions on nutritional therapy in cystic fibrosis, which were divided into six sections: nutritional assessment, nutritional recommendations, nutritional intervention, dietary counseling, special situations and enzyme replacement, and gastrointestinal manifestations. Two to three specialists in the field were responsible for each section and obtaining answers formulated based on standardized bibliographic searches. The available literature was searched in the PubMed®/MEDLINE database, after training and standardization […]

ABSTRACT Objective To describe the drug utilization profile used by pediatric cystic fibrosis patients. Methods A transversal study comprising the analysis of records and interviews with caregivers of pediatric patient in a reference center of Southern Brazil. We collected information about patients’ clinical condition, medication used and household therapy. Results Out of 78 patients participating in the study, prevailing characteristics were: female, self-declared white color, mutation F508del and countryside resident. Forty-three patients had health monitoring exclusively in the hospital’s outpatient […]

Abstract Objective We evaluated the association between vitamin D levels and nutritional status, pulmonary function and pulmonary exacerbations in children and adolescents with cystic fibrosis. Methods 25-hydroxyvitamin D (25(OH)D) levels of 37 children and adolescents were retrospectively evaluated. Pulmonary function, body mass index, height for age, and pulmonary exacerbations episodes were associated with vitamin D levels divided into two groups: sufficient (≥30ng/mL) and hypovitaminosis (

Cystic fibrosis is an autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. This disorder produces a variable phenotype including lung disease, pancreatic insufficiency, and meconium ileus plus bilateral agenesis of the vas deferens causing obstructive azoospermia and male infertility. Preimplantation genetic diagnosis is an alternative that allows identification of embryos affected by this or other genetic diseases. We report a case of couple with cystic fibrosis; the woman had the I148 T mutation and […]