Age-dependent Oral Manifestations of Neurofibromatosis Type 1: A Case-control Study

Abstract

Introduction: Craniofacial manifestations of neurofibromatosis type 1 (NF1) are considered as a result of tumor compression. We sought to determine age-dependent salivary changes, carious, and periodontal complications in NF1 patients without tumors in the oral cavity.Objective and methods: Eleven NF1 patients without tumors in the oral cavity and 29 matched controls without NF1 were enrolled in this case-control study. Demographic information, medical history, and data of intraoral examinations, including the Decayed, Missing, and Filled Teeth (DMFT) scores and Russel’s periodontal index (PI), were recorded. The functional salivary analysis was performed for sialometry, salivary pH values, and amylase activity. Ingenuity Systems Pathway Analysis (IPA) was conducted to identify mutually activated pathways for NF1-associated oral complications.Results: NF1 patients were associated with periodontitis (OR=1.40, 95% CI=1.06-1.73, P = 0.04), gingivitis (OR=1.55, 95% CI=1.09-2.01, P = 0.0002), and decreased salivary flow rate (OR=1.40, 95% CI=1.05-1.76, P = 0.005). Periodontal destruction, salivary changes, and dental caries in NF1 patients were age dependent. Subgroup analyses based on age stratification suggested that salivary flow rates and salivary amylase activity were significantly lower among NF1 patients aged over 20 years and that salivary pH values, PI and DMFT scores were higher among NF1- controls aged over 20. All oral complications were not significantly presented among NF1 patients aged below 20. IPA analyses suggested that cellular mechanisms underlying NF1-associated oral complications involved chronic inflammatory pathways as well as fibrosis signalling pathway.Conclusion: NF1 patients without tumors in the oral cavity presented with a higher prevalence of age-dependent oral complications, including periodontal destruction and salivary gland dysfunction, which were associated with chronic inflammatory pathogenesis.

The clinical relevance and pathogenesis of microbes in ankylosing spondylitis

“…13,14 Periodontal pathogens possess arginine protease (PAD) and research indicates that arginine is crucial for their interactions with HLA-B27. 4 Thus, it has been provided that if an HLA-B27+ individual is infected with Porphyromonas gingivalis PADs, 7,12,[15][16][17][18][19][20] it can trigger a cytotoxic T-cell-mediated autoimmune response that is specific to HLA-B27, leading to inflammation in the joints. 4 Likewise, it has been demonstrated that the expression of HLA-B27 is linked to changes in the gut microbiota, and the difference in the abundance of certain species in gut microbiota between AS patients and healthy individuals is significant.…”