Cystic fibrosis: An updated comprehensive review of literature with pediatric dentistry perspectives

Abstract

Cystic fibrosis (CF) is a genetic disorder, inherited in an autosomal recessive manner. It affects the multiple organs or organ systems of human body including mainly respiratory, digestive, and renal systems. CF patients may have various orodental problems either directly by the primary disease of CF or indirectly by comorbidities, its complications and by side effects of pharmacotherapies. The optimization of oral health by meticulous dental care is essential aspect and hence must be an integral part of the multidisciplinary management in such patients with CF. Thus, general and pediatric dentist need to remain updated with the pathophysiology of the disease, its medical treatment, and its adverse effects on oral health in order to manage such CF-affected individuals with best scientific evidence-based approach of dentistry. Therefore, the current article has comprehensively reviewed the existing current literature regarding CF to describe its pathophysiology (etiology), clinical features, screening/diagnostic modalities, and management strategies along with its detailed orodental implications of CF and dental management aspects. Moreover, an overview/outline of dental care of CF patients in age-wise manner has been illustrated in this article. The relevant and recent scientific literature for the comprehensive review has been searched using the suitable keywords or its combinations/Boolean operators in different electronic databases (PubMed-Medline, Web of Science, Scopus, and Google Scholar) and, thus, included the topic-related articles published in English literature till 2022. Furthermore, the articles related to orodental implications and the dental management of the CF with the perspectives of pediatric dentistry was also searched and included for review.