Activities per year
Abstract
People with PKU are at risk of essential fatty acid deficiency (EFAD) due to dietary restriction of high EFA sources in the treatment diet and inadequate supplementation in some prescribed PKU formulas. Barriers such as poor formula compliance, and periods of rapid growth can heighten the EFAD risk. This investigation aims to identify the proportion of adults with PKU who have EFAD and to detect variables that are associated with EFAD.
Retrospective pathology data was collected from 2011-2017 for adults with PKU. Results retrieved included EFA profiles, with mean blood phenylalanine from 6- and 12- months prior to EFA collection to assess compliance to the PKU treatment diet and formula. Data on age, gender, pregnancy status, anthropometry, formula type and dose were also obtained. Data was collated and analysed using Chi-square tests to identify if differences in EFAD exist across variables.
Full data sets were collected for 124 EFA samples from 51 adults with PKU. Of these EFA samples, 27% (n=33) showed one or more EFAD. Further data analysis revealed that 36.4% (n=12) of the identified EFAD were from pregnant women. Of these pregnancies 76% (n=9) were compliant with prescribed dose of PKU formula and protein restricted diet.
Despite pregnant women with PKU maintaining high compliance with low protein diet and formula, EFAD do readily exist during this rapid growth phase. This investigation stimulates identification and improved dietary management of EFAD in adults with PKU and encourages further research in this area.
Retrospective pathology data was collected from 2011-2017 for adults with PKU. Results retrieved included EFA profiles, with mean blood phenylalanine from 6- and 12- months prior to EFA collection to assess compliance to the PKU treatment diet and formula. Data on age, gender, pregnancy status, anthropometry, formula type and dose were also obtained. Data was collated and analysed using Chi-square tests to identify if differences in EFAD exist across variables.
Full data sets were collected for 124 EFA samples from 51 adults with PKU. Of these EFA samples, 27% (n=33) showed one or more EFAD. Further data analysis revealed that 36.4% (n=12) of the identified EFAD were from pregnant women. Of these pregnancies 76% (n=9) were compliant with prescribed dose of PKU formula and protein restricted diet.
Despite pregnant women with PKU maintaining high compliance with low protein diet and formula, EFAD do readily exist during this rapid growth phase. This investigation stimulates identification and improved dietary management of EFAD in adults with PKU and encourages further research in this area.
Original language | English |
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Publication status | Unpublished - 4 Sept 2018 |
Event | Building Bridges: 42nd Human Genetics Society of Australasia Special Interest Group Meetings: Australasian Society for Inborn Errors of Metabolism - Sydney, Australia Duration: 4 Aug 2018 → 7 Aug 2018 Conference number: 42nd https://www.hgsa.org.au/about/HGSA2018-sig-meetings |
Conference
Conference | Building Bridges: 42nd Human Genetics Society of Australasia Special Interest Group Meetings |
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Abbreviated title | HGSA SIG ASIEM |
Country/Territory | Australia |
City | Sydney |
Period | 4/08/18 → 7/08/18 |
Internet address |
Fingerprint
Dive into the research topics of 'Essential Fatty Acid Deficiencies in Adult PKU patients on low protein diets'. Together they form a unique fingerprint.Related Activities
- 1 Non-HDR Student Supervision
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Gabrielle Beardwood - Masters of Nutrition and Dietetic Practice research project
Gabrielle Beardwood (Student ), Clare Williams (Supervisor), Jim McGill (Supervisor) & Barbara Van der Meij (Supervisor)
1 Jan 2018 → 1 Aug 2018Activity: Professional Development, Mentorship, Supervision and Other Activities › Non-HDR Student Supervision
Related Research Outputs
- 1 Article
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Nutrition process improvements for adult inpatients with inborn errors of metabolism using the i-PARIHS framework
Williams, C., van der Meij, B. S., Nisbet, J., Mcgill, J. & Wilkinson, S. A., Apr 2019, In: Nutrition and Dietetics. 76, 2, p. 141-149 9 p.Research output: Contribution to journal › Article › Research › peer-review
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