title: The oral health needs of children, adolescents and young adults affected by a mucopolysaccharide disorder.
abstract: BACKGROUND
  As one of the 4 United Kingdom national centres for Lysosomal Storage Disorders, Birmingham Children's Hospital (BCH) cares for 49 patients with a mucopolysaccharide (MPS) disorder. Presently, their oral health needs have never been fully investigated. Enzyme therapies are revolutionising medical treatment, and will prolong life expectancy. Therefore, oral health and its impact on quality of life are increasingly important.
  
  
  OBJECTIVE
  Patients with an MPS disorder have greater oral health needs in comparison with the general population.
  
  
  METHODS
  Forty-six patients with an MPS disorder were investigated for caries, gingival health and enamel opacities. Questionnaires to establish the utilisation of dental services and assess the perceived difficulties in achieving adequate oral health care were completed.
  
  
  RESULTS
  Patients with an MPS disorder have specific oral health needs depending on the type of MPS experienced. This study found 76% of patients with MPS IV had experienced dental caries and they all showed evidence of a generalised unspecified enamel defect. Subjects with MPS I, II and III did not have an increased caries rate; 50% of the study group had received dental treatment in the past, of which 74% (17, n = 23) required a general anaesthetic.
  
  
  CONCLUSIONS
  The MPS disorders can have a significant effect on the developing dentition. Subjects with MPS IV do have an increased oral health need in comparison with the general population and the other MPS groups due to their increased caries rate and enamel defects. Further research is required to fully assess their needs and investigate any structural tooth defects.
authors: Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK, Alison.James@bch.nhs.uk.
authors: Alison
authors: A
authors: James
authors: Christian J
authors: CJ
authors: Hendriksz
authors: Owen
authors: O
authors: Addison