The Prevalence of Developmental Defects of Enamel in People with Cystic Fibrosis: A Systematic Review

Abstract

Background Oral health impacts systemic health, individual well-being, and quality of life. It is important to identify both modifiable and non-modifiable risk factors that may impact oral health at an individual and a population level, doing so may aid public health and policy development, and promote targeted patient treatment strategies. Developmental defects both quantitative and qualitative can negatively impact oral health. Affected individuals may be more susceptible to dental caries, hypersensitivity premature tooth wear, erosion, and poor aesthetics. As part of a wider study assessing oral health in adults with cystic fibrosis currently underway in Cork University Dental School and hospital, it was decided to conduct a systematic review of available literature to assess the prevalence of enamel defects in people with cystic fibrosis. Keywords Cystic Fibrosis, Developmental Defects of Enamel, Oral Health, Public Health, Policy, Prevention. Aims To critically evaluate the literature to determine if the prevalence of developmental defects of enamel is higher in people with cystic fibrosis. Methods Data Sources: A systematic review of articles published in the English language from 1960 to 2023 was conducted in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analysis (PRISMA) Statement. Three online databases were searched Embase, Scopus and Web of Science Core Collection. Studies that examined an association between cystic fibrosis and developmental defects of enamel were included in this systematic review. Results Ninety-one studies were identified following a search of online databases and bibliographies. Following application of inclusion and exclusion criteria, and screening by two examiners nine studies were deemed eligible for inclusion in the systematic review. Eight studies reported a higher prevalence of developmental defects of enamel in people with cystic fibrosis, although not all reported differences were statistically significant. One study reported no difference between the study and control group Conclusions The results of the systematic review indicate varying degrees of heterogeneity between studies. However, there is a consistent pattern that the prevalence of DDE is higher in people with cystic fibrosis compared to people without cystic fibrosis. The aetiology of developmental defect of enamel in people with cystic fibrosis has not been determined although genetic dysfunction, chronic systemic infections, and long-term antibiotic use are some of the theorised causes. This systematic review shows the paucity of information regarding the oral health of this vulnerable cohort and highlights the need for future studies to investigate if the disease process of cystic fibrosis is causative factor for developmental defects of enamel.