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BRONJ occurred significantly in patients who underwent dental extractions after the initiation of ZA and did not accomplish a preventive dental program.

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Background The influence of dental treatment on oral health-related quality of life (OHRQOL) has rarely been evaluated in patients with intellectual disability (ID) through validated questionnaires. The aim of this study was to estimate the changes on OHRQOL in patients with ID after the implementation of an institutional dental treatment program under general anesthesia using the Franciscan Hospital for Children Oral Health-Related Quality of Life questionnaire (FHCOHRQOL-Q). Material and Methods A prospective longitudinal study was conducted on 85 patients (mean age=24.85 years) classified according to DSM-V whose parents/caregivers completed the FHC-OHRQOL-Q. We analyzed the changes in the questionnaire’s overall score and its dimensions from pre-treatment to 12-months of follow-up, considering effect sizes and minimal important differences estimated by the standard measurement error. The impact of clinical and therapeutic factors was evaluated using univariate and multiple linear regression analysis ( p <0.05). Results Significant improvement of OHRQOL was found after dental treatment in oral symptoms ( p 0.001), daily life problems ( p =0.018), parent’s perceptions ( p =0.013) and FHCOHRQOL-Q´s overall score ( p =0.001). OHRQOL changes exhibited an intermediate magnitude (0.38-0.21) as estimated by effect sizes. Changes in oral symptoms showed positive correlation with DMFT index ( r =0.375, p =0.002), decayed teeth ( r =0.244, p =0.036), dental extractions ( r =0.424, p <0.001) and number of treatments ( r =0.255, p =0.019). The improvement was greater in patients with 4 decayed teeth ( p =0.049) and undergoing 2 dental extractions ( p =0.002). Multiple regression analysis demonstrated that dental extractions ( p <0.001) and DMFT index ( p =0.028) were significantly related to oral symptom improvement. Conclusions Dental treatment under general anesthesia showed a positive effect on the overall FHC-OHRQOL-Q score and most of its dimensions. At 12-months of follow-up, the improvement of oral symptoms was significantly associated with DMFT index, decayed teeth, dental extractions and number of treatments. In our clinical setting, the implementation of a dental treatment program enhanced the OHRQOL of patients with ID. Key words: Oral health-related quality of life, intellectual disability, general anesthesia, special needs, dental treatment, Franciscan Hospital for Children Oral Health-Related Quality of Life questionnaire.

Case ReportsHMB-45 (>75%), Melan-A (>75%), zonal necrosis, increased Ki-67 index (12%), and elevated mitotic index (3/10 HPF). However, no gene mutations were identified. In the authors' current case, there were areas of strong positive staining of HMB-45 (>75%) and Melan-A (50%), but no necrosis and a low Ki-67 (1%) and mitotic index (1/10 HPF). In the current molecular study, a GNA11 mutation was identified suggesting the possible role of GNA11 mutation in aggressive melanocytoma behavior.Recent studies have attempted to characterize the pathogenetic of meningeal melanocytoma. Numerous gene mutations in meningeal melanocytoma have been reported: GNAQ (47.6%), GNA11 (17.8%), EIF1AX (27.8%), SF3B1 (10%), BAP1 (10%), KRAS (5%), BRAF (3.6%), and NRAS (1.4%). 8 No mutations have been seen in the oncogenic genes c-Kit (0%), HRAS (0%) and the TERT promoter (0%). 8 In some tumors with heterogenous composition, different tumor mutations were present in the more pigmented or less pigmented section. 9 As the orbital component of the reported case was not biopsied, the question remains as to whether the intracranial and intraorbital tumors have identical genomic profiles.Clinicians and investigators have begun to focus on the role of the G-protein genes GNAQ and GNA11 in melanocytic tumors due to the relatively high frequency of mutations in these genes. 5,10 GNAQ and GNA11 are closely related proteins of the Gq family and are critical players in several signaling pathways, including the MAPK pathway. 11 Most published studies focus on the role of GNAQ/GNA11 mutations in other melanocytic lesions-particularly blue nevi and uveal melanomarather than melanocytoma. 12 GNA11 mutations are common in locally advanced primary uveal melanoma and uveal melanoma metastases. 12 Few studies have looked at the prognosis of GNAQ and GNA11 mutations in melanocytoma. 13 van de Nes et al 13 reported that, of the 19 melanocytic tumors studied, all 3 tumors possessing GNA11 mutations were intermediate-grade melanocytomas that recurred, suggesting that GNA11 mutations might be associated with a clinically aggressive phenotype and serve as a marker of prognosis. In meningeal melanocytomas, few mutations in GNA11 have been identified: c.626A > T (exon 5), c.626A > C (exon 5), and codon Q209, not otherwise specified. [14][15][16][17] To the authors' knowledge, this is the first case of a GNA11 Q209L mutation associated with an intracranial melanocytoma and the subsequent development of an intraorbital lesion in a patient with an ipsilateral Nevus of Ota.Nevus of Ota and melanocytomas arise from a common developmental pathway and may carry mutations in GNAQ or GNA11. Mutations in GNA11, such as GNA11 Q209L , are of particular interest as they may have prognostic value. The authors report a GNA11 mutation in a consecutive intracranial and intraorbital melanocytoma in a patient with ipsilateral Nevus of Ota. Further work is needed to determine the prognostic value of GNA11 mutations found in melanocytomas and whether to alter serial monitoring ...