{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T08:41:06Z","timestamp":1788424866057,"version":"build-2803163510"},"reference-count":24,"publisher":"IOP Publishing","issue":"1","license":[{"start":{"date-parts":[[2019,10,21]],"date-time":"2019-10-21T00:00:00Z","timestamp":1571616000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/publishingsupport.iopscience.iop.org\/iop-standard\/v1"},{"start":{"date-parts":[[2019,10,21]],"date-time":"2019-10-21T00:00:00Z","timestamp":1571616000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/iopscience.iop.org\/info\/page\/text-and-data-mining"}],"content-domain":{"domain":["iopscience.iop.org"],"crossmark-restriction":false},"short-container-title":["J. Breath Res."],"published-print":{"date-parts":[[2020,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    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. 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 seventh day, seven exhaled breath samples were collected over a 10 h period. Subjects then transferred to the other PS for a week and on day seven, provided seven 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                    <jats:italic>n<\/jats:italic>\n                    \u00a0=\u00a03) 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.\n                  <\/jats:p>","DOI":"10.1088\/1752-7163\/ab4097","type":"journal-article","created":{"date-parts":[[2019,9,2]],"date-time":"2019-09-02T19:00:19Z","timestamp":1567450819000},"page":"016001","update-policy":"https:\/\/doi.org\/10.1088\/crossmark-policy","source":"Crossref","is-referenced-by-count":8,"title":["Investigation of paediatric PKU breath malodour, comparing glycomacropeptide with phenylalanine free L-amino acid supplements"],"prefix":"10.1088","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4303-6225","authenticated-orcid":false,"given":"Akira","family":"Tiele","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Daly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John","family":"Hattersley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alex","family":"Pinto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon","family":"Evans","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine","family":"Ashmore","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anita","family":"MacDonald","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James A","family":"Covington","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"266","published-online":{"date-parts":[[2019,10,21]]},"reference":[{"key":"jbrab4097bib1","doi-asserted-by":"publisher","first-page":"255","DOI":"10.1037\/neu0000337","type":"journal-article","article-title":"Cognitive outcomes in early-treated adults with phenylketonuria (PKU): a comprehensive picture across domains","volume":"31","author":"Palermo","year":"2017","journal-title":"Neuropsychology"},{"key":"jbrab4097bib2","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1016\/j.ymgme.2009.09.005","type":"journal-article","article-title":"Management of phenylketonuria in Europe: survey results from 19 countries","volume":"99","author":"Blau","year":"2010","journal-title":"Mol. 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All rights, including for text and data mining, AI training, and similar technologies, are reserved.","name":"copyright_information","label":"Copyright Information"},{"value":"2019-07-05","name":"date_received","label":"Date Received","group":{"name":"publication_dates","label":"Publication dates"}},{"value":"2019-09-02","name":"date_accepted","label":"Date Accepted","group":{"name":"publication_dates","label":"Publication dates"}},{"value":"2019-10-21","name":"date_epub","label":"Online publication date","group":{"name":"publication_dates","label":"Publication dates"}}]}}