Abstract
Down syndrome (DS) is an autosomal disorder associated with mental and physical involvement. The typical craniofacial phenotype and the dental anomalies in DS subjects have been widely described, but a systematic report on the manifestations affecting the oral mucosae in children with DS is still lacking. This systematic review aimed to establish the prevalence of oral mucosal manifestations in children/young adults with DS. Pubmed, Web of Science, and Scopus were investigated in September 2020. Documents in English on DS children/young adults (up to 25 years) reporting oral mucosal findings were considered. Study quality was assessed with ROBIN-I. Of the 150 references retrieved, 14 studies were considered eligible. The risk of bias ranged from low to unclear. Fissured tongue appeared to increase with age and was more prevalent in DS children than in the general population. Lip fissures and cheilitis were heterogeneously reported. Candida spp. carriage with and without active candidiasis was more frequent in DS children/young adults than in controls. C. albicans was the most prevalent species. Few other oral mucosal conditions have been reported sporadically. The heterogeneity of the works revealed the need for more appropriate oral examination to intercept the oral manifestations of oral mucosa and prevent recurrent candidiasis.
Highlights
Down syndrome (DS) is an autosomal congenital disorder caused by the presence of all or part of an extra 21st chromosome
The protocol was registered on PROSPERO and the research query was formulated according to the PICOS method (Participants, Intervention, Comparison, Outcomes, and Study): the participants were children and young adults affected by Down syndrome (DS); the intervention was the examination of the oral mucosa; the comparison was towards subjects not affected by DS; the outcomes included the incidence/prevalence of oral mucosal manifestations; and the study design was a systematic review
After 73 duplicates were removed, the remaining 150 articles underwent the first selection based on inclusion/exclusion criteria and title/abstract
Summary
Down syndrome (DS) is an autosomal congenital disorder caused by the presence of all or part of an extra 21st chromosome. DS is called Trisomy 21, 95% of cases being caused by a numerical change of chromosome 21, with an extra copy that creates a chromosome count of 47; less frequently, DS is a consequence of the translocation of part of an extra chromosome 21 (3–4% of cases), or mosaicisms (1–2% of cases) [1]. DS is the most common trisomy compatible with life; its incidence is influenced by maternal age and ranges from 1 in 319 to 1000 live births worldwide [2]. The most common dermatological manifestations include alopecia areata, premature hair greying and ageing, palmoplantar hyperkeratosis, and xerosis [3]
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