Oral health in individuals with cystic fibrosis: A cross-sectional study.

Cystic Fibrosis (CF) is an autosomal recessive disease characterized by the production of thick and viscous mucus progressively affecting various organs and systems, with recurrent respiratory infections. The aim of this study was to learn about the oral health characteristics in CF patients. METHODOLOGY: Data, such as sociodemographic, general and oral health, were collected from the medical records of CF patients aged 0 to 18 years old. The number of patients with tooth decay, prevalence of developmental defects of enamel (DDE), classification of dental occlusion, sialometry, salivary pH and oral microbial profile and respiratory secretions evaluations were recorded. RESULTS: Most patients had pancreatic insufficiency (84.2%), malnutrition (60%), respiratory problems (75.4%) and genotyping of the F508del (66.7%). Regarding the medications used, 96.5% used vitamins and electrolyte replacement, 84,02% used pancreatic enzymes, 64.9% used dornase alfa and 47% were using antibiotics. The percentage of patients with tooth decay was 19.3%, 47% had DDE, low salivary flow and basic salivary pH. The most prevalent microorganisms found on tongue biofilm and respiratory secretions were SA and PA. There was a positive association between the presence of bacteria and fungi found on both the tongue and respiratory secretions. The presence of fungi on the tongue biofilm was significantly associated with the use of antibiotics. CONCLUSIONS: These findings underscore the importance of dentists focusing on prevention and on the specific needs of the patient as well.

Cystic Fibrosis: Clinical Phenotypes in Children and Adolescents.

PURPOSE: The objective of this study was to describe the clinical phenotypes of children and adolescents with cystic fibrosis (CF); and to assess the role of pancreatic insufficiency and neonatal screening in diagnosis. METHODS: A cross-sectional study was conducted, which included 77 patients attending a reference center of CF between 2014 and 2016. Epidemiological data, anthropometric measurements, and the presence of pulmonary, pancreatic, gastrointestinal and hepatobiliary manifestations were evaluated based on clinical data and complementary examinations. RESULTS: Of the 77 patients, 51.9% were male, with a median age of 147 months (7.0-297.0 months), and the majority showed adequate nutritional status. The most common phenotype was pulmonary (92.2%), followed by pancreatic (87.0%), with pancreatic insufficiency in most cases. Gastrointestinal manifestation occurred in 46.8%, with constipation being the more common factor. Hepatobiliary disease occurred in 62.3% of patients. The group with pancreatic insufficiency was diagnosed earlier (5.0 months) when compared to the group with sufficiency (84.0 months) (p=0.01). The age of diagnosis was reduced following implementation of neonatal screening protocols for CF (6.0 months before vs. 3.0 months after, p=0.02). CONCLUSION: The pulmonary phenotype was the most common, although extrapulmonary manifestations were frequent and clinically relevant, and should mandate early detection and treatment. Neonatal screening for CF led to earlier diagnosis in patients with pancreatic failure, and therefore, should be adopted universally.