Enamel Renal Syndrome: A Scoping Review Protocol

Abstract

Background: Enamel Renal Syndrome (ERS) (OMIM # 204690) is a rare autosomal recessive disorder characterized by hypoplastic amelogenesis imperfecta (AI), failed tooth eruption, intra-pulpal calcifications, gingival enlargement and nephrocalcinosis. The rarity of the condition and the variability of the phenotype has led to ERS not being fully characterized. This scoping review aims to account for the range and current state of knowledge on ERS and synthesize these findings into a comprehensive summary, focusing on the pathophysiology, genotype-phenotype correlations and patient management from a dental perspective.Methods: The authors will conduct a systematic search of PubMed (MEDLINE), BioMed Central, EbscoHost Web, Web of Science and WorldCat. We will include all studies with human participants with a confirmed diagnosis of ERS. Articles will be screened in two stages i.e. initially by title and abstract screening and then full-text screening by two independent reviewers. Data extraction will be conducted using a customised electronic data extraction form. We will provide a narrative synthesis of the findings from the included studies. We will structure the results according to themes.Discussion: Dentists should be able to identify patients with clinical features of ERS so that they receive appropriate referrals for renal evaluation, genetic counselling and oral rehabilitation to increase the patient’s quality of life. A scoping review is the most appropriate method to conduct this comprehensive exploration of the current evidence which may be sparse due to the rarity of the condition. It will also enable us to identify gaps in the research.Registration: This study is registered with the Open Science Framework (OSF) (https://osf.io/cghsa).

Enamel Renal Syndrome: Protocol for a Scoping Review

“…There was no patient participation in this study. This protocol was completed in July 2020 and registered in OSF [ 10 ] in August 2020. The electronic search for literature was undertaken in July 2020 and updated in April 2021.…”

“…Reviews and Meta-Analysis Extension for Scoping Reviews) guidelines [9]. This study is registered with the Open Science Framework (OSF) [10].…”

“…We will undertake this scoping review following the PRISMA-ScR (Preferred Reporting Items for Systematic Reviews and Meta-Analysis Extension for Scoping Reviews) guidelines [ 9 ]. This study is registered with the Open Science Framework (OSF) [ 10 ].…”

Escalating the Importance of Preserving the Electronic Records Metadata in Online Business Transaction Activities

“…According to Guden & Bellen (2020), the scoping review is an effective means of highlighting the relevant literature to the researcher, with the aim of rapid mapping of key concepts underpinning a research area and a robust method of identifying primary and secondary sources of literature. Significantly, as stated by Roomaney et al (2020), the scoping review is the most appropriate method to conduct this comprehensive exploration of the current evidence which may be sparse due to the rarity of the condition. Based on the discussions, the selection of scoping review technique in this paper is firmly justified.…”