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A review of craniofacial and dental findings of the RASopathies #MMPMID28643916
Cao H; Alrejaye N; Klein OD; Goodwin AF; Oberoi S
Orthod Craniofac Res 2017[Jun]; 20 (Suppl 1): 32-8 PMID28643916show ga
Objectives: The RASopathies are a group of syndromes that have in common germline mutations in genes that encode components of the Ras/mitogen-activated protein kinase (MAPK) pathway and have been a focus of study to understand the role of this pathway in development and disease. These syndromes include Noonan syndrome (NS); Noonan syndrome with multiple lentigines (NSML or LEOPARD syndrome); neurofibromatosis type 1 (NF1); Costello syndrome (CS); cardio-facio-cutaneous (CFC) syndrome; neurofibromatosis type 1-like syndrome (NFLS or Legius syndrome); and capillary malformation-arteriovenous malformation syndrome (CM-AVM). These disorders affect multiple systems, including the craniofacial complex. Although the craniofacial features have been well described and can aid in clinical diagnosis, the dental phenotypes have not been analyzed in detail for each of the RASopathies. In this review, we summarize the clinical features of the RASopathies, highlighting the reported craniofacial and dental findings. Methods: Review of the literature. Results: Each of the RASopathies reviewed, caused by mutations in genes that encode different proteins in the Ras pathway, have unique and overlapping craniofacial and dental characteristics. Conclusions: Careful description of craniofacial and dental features in the RASopathies can provide information for dental clinicians treating these individuals and can also give insight into the role of Ras signaling in craniofacial development.