A Systematic Review of the Prevalence of Developmental Defects of Enamel in People With Cytsic Fibrosis
Objectives: To critically evaluate the literature on the prevalence of developmental defects of enamel in people with cystic fibrosis, in order to establish if this group are at increased risk of developmental defects of enamel.
Methods: Three online databases – Embase, Web of Science Core Collection, and Scopus– were used to identify relevant references and citations using a combination of the following search terms: “cystic fibrosis”, “dental hard tissue”, “enamel defect”, “developmental enamel defect”, “dental hypoplasia”, “dental hyperplasia”, and “oral health”. A systematic review of English language articles from 1960 to 2020, in accordance with the Preferred Reporting Items for Systematic reviews and Meta-Analysis(PRISMA) Statement was conducted. Studies that were of case-control or cohort or cross sectional design were included. Studies were included if they directly examined an association between cystic fibrosis and developmental defects of enamel.
Results: The search resulted in 123 publications from Embase, Scopus and Web of Science Core Collection. Based on information provided in the study title and abstract, 114 publications were excluded. Articles with an irrelevant subject topic, and systematic or literature reviews were excluded. Nine publications met eligibility criteria and were included in this systematic review.
Conclusions: There is a general finding that the prevalence of DDE is higher in people with Cystic Fibrosis. Eight studies reported an increased prevalence of DDE in study groups compared to non-Cystic Fibrosis control groups. Not all differences were statistically significant. One study reported no difference between the study and control group. There is a lack of consensus on the aetiology of DDE in people with Cystic Fibrosis, however, dysfunction of the Cystic Fibrosis Transmembrane Conductance Regulator gene, chronic systemic infection and long-term antibiotic use have been theorised as possible causes. There is a need for future studies to investigate if the disease process of Cystic Fibrosis is a causative factor for DDE.