Creatine Levels in Patients with Phenylketonuria and Mild Hyperphenylalaninemia: A Pilot Study
Background: Creatine (Cr) levels are strongly dependent on diets, including animal-derived proteins. Cr is an important metabolite as it represents a source of stored energy to support physical performance and potentially sustain positive effects such as improving memory or intelligence. This study...
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MDPI AG
2021-05-01
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| Online Access: | https://www.mdpi.com/2075-1729/11/5/425 |
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| author | Elvira Verduci Maria Teresa Carbone Laura Fiori Claudia Gualdi Giuseppe Banderali Claudia Carducci Vincenzo Leuzzi Giacomo Biasucci Gian Vincenzo Zuccotti |
| author_facet | Elvira Verduci Maria Teresa Carbone Laura Fiori Claudia Gualdi Giuseppe Banderali Claudia Carducci Vincenzo Leuzzi Giacomo Biasucci Gian Vincenzo Zuccotti |
| author_sort | Elvira Verduci |
| collection | DOAJ |
| description | Background: Creatine (Cr) levels are strongly dependent on diets, including animal-derived proteins. Cr is an important metabolite as it represents a source of stored energy to support physical performance and potentially sustain positive effects such as improving memory or intelligence. This study was planned to assess Cr levels in PKU children adhering to a diet low in phenylalanine (Phe) content and compared with those of children with mild hyperphenylalaninemia (MHP) on a free diet. Methods: This retrospective pilot study analyzed Cr levels from Guthrie cards in 25 PKU and 35 MHP subjects. Anthropomorphic and nutritional data of the study populations were assessed, compared and correlated. Results: Cr levels of PKU subjects were significantly lower than those of MHP subjects and correlated to the low intake of animal proteins. Although no deficiencies in PKU subjects were identified, PKU subjects were found to have a 26-fold higher risk of displaying Cr levels <25° percentile than MHP counterparts. Conclusions: This pilot study suggests that Cr levels might be concerningly low in PKU children adhering to a low-Phe diet. Confirmatory studies are needed in PKU patients of different age groups to assess Cr levels and the potential benefits on physical and intellectual performance of Cr supplementation. |
| first_indexed | 2024-03-10T11:39:54Z |
| format | Article |
| id | doaj.art-7df49396506a4c8dab9ca78cd5f0fafe |
| institution | Directory Open Access Journal |
| issn | 2075-1729 |
| language | English |
| last_indexed | 2024-03-10T11:39:54Z |
| publishDate | 2021-05-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Life |
| spelling | doaj.art-7df49396506a4c8dab9ca78cd5f0fafe2023-11-21T18:35:16ZengMDPI AGLife2075-17292021-05-0111542510.3390/life11050425Creatine Levels in Patients with Phenylketonuria and Mild Hyperphenylalaninemia: A Pilot StudyElvira Verduci0Maria Teresa Carbone1Laura Fiori2Claudia Gualdi3Giuseppe Banderali4Claudia Carducci5Vincenzo Leuzzi6Giacomo Biasucci7Gian Vincenzo Zuccotti8Department of Pediatrics, Vittore Buzzi Children’s Hospital-University of Milan, Via Lodovico Castelvetro, 32, 20154 Milan, ItalyUOS Metabolic and Rare Diseases, AORN Santobono, Via Mario Fiore 6, 80122 Naples, ItalyDepartment of Pediatrics, Vittore Buzzi Children’s Hospital-University of Milan, Via Lodovico Castelvetro, 32, 20154 Milan, ItalyPediatric Division, ASST Grande Ospedale Metropolitano Niguarda, Piazza Ospedale Maggiore 3, 20162 Milan, ItalyPediatric Unit, San Paolo Hospital, ASST Santi Paolo e Carlo Hospital, Via di Rudinì 8, 20142 Milan, ItalyDepartment of Experimental Medicine, Sapienza University of Rome, Viale Regina Elena 324, 00161 Rome, ItalyDepartment of Human Neuroscience, Sapienza University of Rome, Via dei Sabelli 108, 00185 Rome, ItalyDepartment of Pediatrics & Neonatology, Guglielmo da Saliceto Hospital, Via Taverna Giuseppe, 49, 29121 Piacenza, ItalyDepartment of Pediatrics, Vittore Buzzi Children’s Hospital-University of Milan, Via Lodovico Castelvetro, 32, 20154 Milan, ItalyBackground: Creatine (Cr) levels are strongly dependent on diets, including animal-derived proteins. Cr is an important metabolite as it represents a source of stored energy to support physical performance and potentially sustain positive effects such as improving memory or intelligence. This study was planned to assess Cr levels in PKU children adhering to a diet low in phenylalanine (Phe) content and compared with those of children with mild hyperphenylalaninemia (MHP) on a free diet. Methods: This retrospective pilot study analyzed Cr levels from Guthrie cards in 25 PKU and 35 MHP subjects. Anthropomorphic and nutritional data of the study populations were assessed, compared and correlated. Results: Cr levels of PKU subjects were significantly lower than those of MHP subjects and correlated to the low intake of animal proteins. Although no deficiencies in PKU subjects were identified, PKU subjects were found to have a 26-fold higher risk of displaying Cr levels <25° percentile than MHP counterparts. Conclusions: This pilot study suggests that Cr levels might be concerningly low in PKU children adhering to a low-Phe diet. Confirmatory studies are needed in PKU patients of different age groups to assess Cr levels and the potential benefits on physical and intellectual performance of Cr supplementation.https://www.mdpi.com/2075-1729/11/5/425PKUmild hyperphenylalaninemialow-Phe dietCrenergyATP |
| spellingShingle | Elvira Verduci Maria Teresa Carbone Laura Fiori Claudia Gualdi Giuseppe Banderali Claudia Carducci Vincenzo Leuzzi Giacomo Biasucci Gian Vincenzo Zuccotti Creatine Levels in Patients with Phenylketonuria and Mild Hyperphenylalaninemia: A Pilot Study Life PKU mild hyperphenylalaninemia low-Phe diet Cr energy ATP |
| title | Creatine Levels in Patients with Phenylketonuria and Mild Hyperphenylalaninemia: A Pilot Study |
| title_full | Creatine Levels in Patients with Phenylketonuria and Mild Hyperphenylalaninemia: A Pilot Study |
| title_fullStr | Creatine Levels in Patients with Phenylketonuria and Mild Hyperphenylalaninemia: A Pilot Study |
| title_full_unstemmed | Creatine Levels in Patients with Phenylketonuria and Mild Hyperphenylalaninemia: A Pilot Study |
| title_short | Creatine Levels in Patients with Phenylketonuria and Mild Hyperphenylalaninemia: A Pilot Study |
| title_sort | creatine levels in patients with phenylketonuria and mild hyperphenylalaninemia a pilot study |
| topic | PKU mild hyperphenylalaninemia low-Phe diet Cr energy ATP |
| url | https://www.mdpi.com/2075-1729/11/5/425 |
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