What are the oral health needs of children with Phenylketonuria (PKU)?
Research type
Research Study
Full title
What are the oral health needs of children with Phenylketonuria (PKU)?
IRAS ID
280067
Contact name
Laura Johnston
Contact email
Sponsor organisation
Birmingham Women`s and Children's Hospital
Duration of Study in the UK
2 years, 0 months, 0 days
Research summary
Phenylketonuria, better known as PKU, is a rare autosomal recessive genetic disorder which causes a raise in phenylalanine (Phe) levels. Treatment consists of maintaining a low blood Phe, achieved via a strict low-phe diet combined with phe-free L-amino acid supplements. Increased frequency and content of sugars in phe-free foods and supplements may increase risk of developing dental caries (decay).
Children with PKU under the care of Birmingham Women’s and Children’s Hospital (BWCH) Metabolic team have been identified as having pain and infection from dental caries. For children with PKU, dental infection can cause catabolism of muscle protein resulting in an elevation of plasma Phe concentrations and poor PKU control.
Previous research surrounding caries experience and oral health practices in children with PKU is limited. Recent studies have found a higher experience of dental caries in the primary dentition and higher levels of acid erosion (tooth wear).
This cross-sectional study will consist of an oral health questionnaire and diet analysis to establish caries risk factors. Review of dental records and previous control of Phe levels will identify any links between PKU control and caries experience. If participants attend BWCH a dental examination will be conducted to allow comparison to the general population via child dental health studies. All children aged 1-16 with PKU under the care of the Department of Metabolic Disease, BWCH, and unaffected siblings, will be invited to take part in the study.
The primary research objective is to establish the caries experience of children with PKU (dmft/DMFT) in comparison to that of the general population (via Child Dental Health Surveys). Subsequent outcomes will help to develop a bespoke dental prevention programme for children with PKU under the care of BWCH Metabolic team.
REC name
South West - Frenchay Research Ethics Committee
REC reference
21/SW/0073
Date of REC Opinion
7 Jul 2021
REC opinion
Further Information Favourable Opinion