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Investigation of paediatric PKU breath malodour, comparing glycomacropeptide with phenylalanine free L-amino acid supplements
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Tiele, Akira, Daly, Anne, Hattersley, John, Pinto, Alex, Evans, Sharon, Ashmore, Catherine, MacDonald, Anita and Covington, James A. (2019) Investigation of paediatric PKU breath malodour, comparing glycomacropeptide with phenylalanine free L-amino acid supplements. Journal of Breath Research . doi:10.1088/1752-7163/ab4097
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Official URL: https://doi.org/10.1088/1752-7163/ab4097
Abstract
In clinical practice, caregivers of children with phenylketonuria (PKU) report that their children have breath malodour. This might be linked to the regular consumption of low phenylalanine (Phe)/Phe-free protein substitutes (PS), which are an essential component of a low-Phe diet. Oral malodour can negatively affect interpersonal communication, lead to bullying, low self-esteem and social isolation. In this longitudinal cross-over study, exhaled volatile organic compounds (VOCs) were measured using gas chromatography - ion mobility spectrometry (GC-IMS). 40 children (20 PKU, 20 controls) were recruited. Subjects with PKU took either L-Amino Acid (L-AA) or Casein Glycomacropeptide (CGMP-AA) exclusively for 1 week, in a randomised order. On the 7th day, 7 exhaled breath samples were collected over a 10-hr period. Subjects then transferred to the other PS for a week and on day 7, provided 7 further breath samples. All subjects had a standardised menu using low-Phe food alternatives and all food intake was measured and recorded. In the PKU group, the aim was to collect samples 30-min after consuming PS. In 3 subjects, breath was collected 5-min post-PS consumption. Fasted L-AA and CGMP-AA breath samples contained a similar number of VOC peaks (10-12) as controls. Longitudinal breath testing results demonstrate that there was no significant difference in the number of exhaled VOCs, comparing L-AA or CGMP-AA with controls, or between PS (12-18 VOC peaks). Breath analysed immediately after consumption of PS (n=3) showed an immediate increase in the number of VOC peaks (25-30), but these were no longer detectable at 30-min post-consumption. This suggests PS have a transient effect on exhaled breath. Measurements taken 30-min after consuming L-AA or CGMP-AA were not significantly different to controls. This indicates that timing food and drinks with PS consumption may be a potential solution for carers to reduce or eliminate unpleasant PS-related breath odours. [Abstract copyright: © 2019 IOP Publishing Ltd.]
| Item Type: | Journal Article | ||||||
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| Subjects: | Q Science > QD Chemistry Q Science > QP Physiology R Medicine > RC Internal medicine R Medicine > RJ Pediatrics |
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| Divisions: | Faculty of Science > Engineering | ||||||
| SWORD Depositor: | Library Publications Router | ||||||
| Library of Congress Subject Headings (LCSH): | Phenylketonurias , Phenylketonurias -- Diagnosis, Bad breath, Gas chromatography, Ion mobility spectroscopy, Volatile organic compounds | ||||||
| Journal or Publication Title: | Journal of Breath Research | ||||||
| Publisher: | IOP Publishing Ltd | ||||||
| ISSN: | 1752-7163 | ||||||
| Official Date: | 2 September 2019 | ||||||
| Dates: |
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| Date of first compliant deposit: | 1 October 2019 | ||||||
| DOI: | 10.1088/1752-7163/ab4097 | ||||||
| Status: | Peer Reviewed | ||||||
| Publication Status: | Published | ||||||
| Access rights to Published version: | Open Access |
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