Investigation of paediatric PKU breath malodour, comparing glycomacropeptide with phenylalanine free L-amino acid supplements

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 alterna...

Source: Journal of Breath Research - October 19, 2019 Category: Respiratory Medicine Authors: Akira Tiele, Anne Daly, John Hattersley, Alex Pinto, Sharon Evans, Catherine Ashmore, Anita MacDonald and James A Covington Source Type: research