title: Synchrotron X-ray diffraction and scanning electron microscopy to understand enamel affected by metabolic disorder mucopolysaccharidosis.
abstract: Mucopolysaccharidosis (MPS) is an inherited metabolic disorder that can affect the tooth structure leading to defects. Synchrotron X-ray diffraction being a state of the art technique has been used to determine the enamel crystallite orientation in deciduous enamel affected by Mucopolysaccharidosis Type I and Mucopolysaccharidosis Type IVA and comparing these with that of healthy deciduous enamel. Using this technique it was observed that there is a loss of texture in deciduous enamel affected by Mucopolysaccharidosis Type I and Mucopolysaccharidosis Type IVA when compared to the healthy deciduous enamel. Generally it was observed that the incisal surface of the deciduous teeth possessed a higher texture or preferred orientation of enamel crystallites and on progression towards the cervical region there was a decrease in the texture or preferred orientation of enamel crystallites. Scanning electron microscopy showed that the presence of a poorly calcified layer between the enamel and dentine at the enamel-dentine junction (EDJ) in MPS affected samples was likely to be responsible for rendering the tooth structure weak and prone to fracture as is often the case in MPS affected deciduous enamel.
authors.affiliation: Queen Mary University of London, Barts and the London School of Medicine and Dentistry, Institute of Dentistry, London E1 4NS, UK. Electronic address: arshman.khan86@gmail.com.
authors.forename: Malik Arshman
authors.initials: MA
authors.lastname: Khan
authors.affiliation: University of Birmingham, School of Dentistry, Birmingham B4 6NN, UK.
authors.forename: Owen
authors.initials: O
authors.lastname: Addison
authors.affiliation: Birmingham Children's Hospital NHS Foundation Trust, Birmingham B4 6NH, UK.
authors.forename: Alison
authors.initials: A
authors.lastname: James
authors.affiliation: Manchester Academic Health Science Centre, The Mark Holland Metabolic Unit, SalfordRoyal Foundation NHS Trust, Ladywell NW2-2nd Floor Room 112, Salford, Manchester M6 8HD, UK.
authors.forename: Christian J
authors.initials: CJ
authors.lastname: Hendriksz
authors.affiliation: Queen Mary University of London, Barts and the London School of Medicine and Dentistry, Institute of Dentistry, London E1 4NS, UK.
authors.forename: Maisoon
authors.initials: M
authors.lastname: Al-Jawad
mesh_terms: D003743:Dental Enamel / Q000737:chemistry* / Q000648:ultrastructure*; D006801:Humans; D008855:Microscopy, Electron, Scanning; D009083:Mucopolysaccharidoses / Q000175:diagnosis*; D014961:X-Ray Diffraction
keywords: Enamel; Morquio syndrome; Mucopolysaccharidosis; Preferred orientation; Scanning electron microscopy; Synchrotron X-ray diffraction