Satisfaction with resilient denture liner versus acrylic resin telescopic prostheses for patients with ectodermal dysplasia: A nonrandomized crossover clinical trial.

STATEMENT OF PROBLEM: Patients with ectodermal dysplasia are characterized by anodontia or oligodontia. How their challenging prosthodontic rehabilitation might be optimized is unclear. PURPOSE: The purpose of this crossover study was to evaluate the effect of resilient denture liner versus acrylic resin copings in complete overdentures for patients with ectodermal dysplasia. Outcome measures included patient satisfaction, retention, and periodontal health of the abutment tooth. MATERIAL AND METHODS: Ten partially edentulous participants diagnosed with ectodermal dysplasia were recruited from the Faculty of Dentistry, Cairo University, Egypt, and enrolled in this crossover clinical trial. All participants received acrylic resin coping-retained maxillary complete overdentures (group N, stage 1). The acrylic resin copings were then replaced by a resilient denture liner (group S, stage 2). Patient satisfaction, retention, and periodontal health parameters were evaluated 1 week and 3 months after the completion of each stage. Patient satisfaction was assessed with a validated, reliable questionnaire. The results of the periodontal probing depths were tested with repeated measures ANOVA followed by the Bonferroni correction for pairwise comparisons. Tooth mobility, patient satisfaction, retention, and gingival index were tested by using the Wilcoxon signed ranked test. Ordinal data as the sixth and seventh domains of patient satisfaction were tested by using the McNemar test for paired comparisons (α=.05). RESULTS: Three months after overdenture delivery, a statistically significant difference was found between the groups regarding retention (P=.025), probing depth (P<.001), and gingival index (P=.011) favoring the acrylic resin coping-retained overdentures. Results of tooth mobility (P=.035), overall attitude (P=.041), ease of eating (P=.023), denture comfort (P=.024), and degree of teasing (P=.038) on wearing the denture showed a statistically significant difference between the groups, favoring the resilient denture liner. CONCLUSIONS: In children with oligodontia and ectodermal dysplasia, the resilient denture liner-retained maxillary complete overdenture enhanced patient satisfaction and tooth mobility of anterior teeth, while minimally jeopardizing the periodontal condition of the abutment teeth.

Gene Mutations of the Three Ectodysplasin Pathway Key Players (EDA, EDAR, and EDARADD) Account for More than 60% of Egyptian Ectodermal Dysplasia: A Report of Seven Novel Mutations.

Ectodermal dysplasia (ED) is a diverse group of genetic disorders caused by congenital defects of two or more ectodermal-derived body structures, namely, hair, teeth, nails, and some glands, e.g., sweat glands. Molecular pathogenesis of ED involves mutations of genes encoding key proteins of major developmental pathways, including ectodysplasin (EDA) and wingless-type (WNT) pathways. The most common ED phenotype is hypohidrotic/anhidrotic ectodermal dysplasia (HED) featuring hypotrichosis, hypohidrosis/anhidrosis, and hypodontia. Molecular diagnosis is fundamental for disease management and emerging treatments. We used targeted next generation sequencing to study EDA, EDAR, EDARADD, and WNT10A genes in 45 Egyptian ED patients with or without hypohidrosis. We present genotype and phenotype data of 28 molecularly-characterized patients demonstrating genetic heterogeneity, variable expressivity, and intrafamilial phenotypic variability. Thirteen mutations were reported, including four novel EDA mutations, two novel EDARADD, and one novel EDAR mutations. Identified mutations congregated in exons encoding key functional domains. EDA is the most common gene contributing to 85% of the identified Egyptian ED genetic spectrum, followed by EDARADD (10%) and EDAR (5%). Our cohort represents the first and largest cohort from North Africa where more than 60% of ED patients were identified emphasizing the need for exome sequencing to explore unidentified cases.