Data on phenylalanine-to-tyrosine ratios in assessment of tetrahydrobiopterin (BH4)-responsiveness in patients with hyperphenylalaninemia
Blood phenylalanine-to-tyrosine (Phe/Tyr) ratio is an important indicator of metabolic control in phenylketonuria patients. We present the data that highlights the role of Phe/Tyr-ratio in the evaluation of tetrahydrobiopterin (BH4)-responsiveness in patients with hyperphenylalaninemia. Our data com...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-04-01
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| Series: | Data in Brief |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S235234092200138X |
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| author | Barbka Repic Lampret Mojca Zerjav Tansek Blaz Groselj Jaka Sikonja Tadej Battelino Urh Groselj |
| author_facet | Barbka Repic Lampret Mojca Zerjav Tansek Blaz Groselj Jaka Sikonja Tadej Battelino Urh Groselj |
| author_sort | Barbka Repic Lampret |
| collection | DOAJ |
| description | Blood phenylalanine-to-tyrosine (Phe/Tyr) ratio is an important indicator of metabolic control in phenylketonuria patients. We present the data that highlights the role of Phe/Tyr-ratio in the evaluation of tetrahydrobiopterin (BH4)-responsiveness in patients with hyperphenylalaninemia. Our data complements the results from the original research article by Tansek et al., 2012 [1]. We performed a BH4-loading test in 32 patients after four days of increased protein intake (2000 mg/kg body weight). Blood sampling was performed 96, 72, 48, 24, 16 h, and moments before oral administration of BH4 in a dose of 20 mg/kg body weight. Additional blood samples were collected 8 and 24 h after its administration. Phenylalanine (Phe) and Tyrosine (Tyr) levels were determined from dried blood spots by tandem mass spectrometry. Phe/Tyr-ratio reached a plateau after three days of increased dietary protein intake. Fifteen patients (47%) responded to BH4, defined as a decrease of Phe-of at least 30% after 24 h of BH4 administration. Phe/Tyr-ratios were significantly higher in non-responders compared to responders. In the responder group, Phe/Tyr-ratios decreased in average of 67% (p = 0.001) and 45% (p = 0.001) after 8 and 24 h of BH4 administration, respectively. Phe/Tyr-ratio decreased after 8 h of drug administration also in the non-responder group, but not 24 h after administration. |
| first_indexed | 2024-12-13T09:02:00Z |
| format | Article |
| id | doaj.art-a4777b2c15f246c4a7c4c6f66cd74867 |
| institution | Directory Open Access Journal |
| issn | 2352-3409 |
| language | English |
| last_indexed | 2024-12-13T09:02:00Z |
| publishDate | 2022-04-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Data in Brief |
| spelling | doaj.art-a4777b2c15f246c4a7c4c6f66cd748672022-12-21T23:53:09ZengElsevierData in Brief2352-34092022-04-0141107926Data on phenylalanine-to-tyrosine ratios in assessment of tetrahydrobiopterin (BH4)-responsiveness in patients with hyperphenylalaninemiaBarbka Repic Lampret0Mojca Zerjav Tansek1Blaz Groselj2Jaka Sikonja3Tadej Battelino4Urh Groselj5Clinical Institute for Special Laboratory Diagnostics, University Children's Hospital, UMC Ljubljana, Ljubljana 1000, SloveniaDepartment of Pediatric Endocrinology, Diabetes and Metabolic Diseases, University Children's Hospital, UMC Ljubljana, Ljubljana 1000, Slovenia; Faculty of Medicine, University of Ljubljana, Ljubljana 1000, SloveniaFaculty of Medicine, University of Ljubljana, Ljubljana 1000, Slovenia; Institute of Oncology Ljubljana, Ljubljana 1000, SloveniaFaculty of Medicine, University of Ljubljana, Ljubljana 1000, SloveniaDepartment of Pediatric Endocrinology, Diabetes and Metabolic Diseases, University Children's Hospital, UMC Ljubljana, Ljubljana 1000, Slovenia; Faculty of Medicine, University of Ljubljana, Ljubljana 1000, SloveniaDepartment of Pediatric Endocrinology, Diabetes and Metabolic Diseases, University Children's Hospital, UMC Ljubljana, Ljubljana 1000, Slovenia; Faculty of Medicine, University of Ljubljana, Ljubljana 1000, Slovenia; Corresponding author at: Department of Pediatric Endocrinology, Diabetes and Metabolic Diseases, University Children's Hospital, UMC Ljubljana, Faculty of Medicine, University of Ljubljana, Bohoriceva 20, Ljubljana 1000, SloveniaBlood phenylalanine-to-tyrosine (Phe/Tyr) ratio is an important indicator of metabolic control in phenylketonuria patients. We present the data that highlights the role of Phe/Tyr-ratio in the evaluation of tetrahydrobiopterin (BH4)-responsiveness in patients with hyperphenylalaninemia. Our data complements the results from the original research article by Tansek et al., 2012 [1]. We performed a BH4-loading test in 32 patients after four days of increased protein intake (2000 mg/kg body weight). Blood sampling was performed 96, 72, 48, 24, 16 h, and moments before oral administration of BH4 in a dose of 20 mg/kg body weight. Additional blood samples were collected 8 and 24 h after its administration. Phenylalanine (Phe) and Tyrosine (Tyr) levels were determined from dried blood spots by tandem mass spectrometry. Phe/Tyr-ratio reached a plateau after three days of increased dietary protein intake. Fifteen patients (47%) responded to BH4, defined as a decrease of Phe-of at least 30% after 24 h of BH4 administration. Phe/Tyr-ratios were significantly higher in non-responders compared to responders. In the responder group, Phe/Tyr-ratios decreased in average of 67% (p = 0.001) and 45% (p = 0.001) after 8 and 24 h of BH4 administration, respectively. Phe/Tyr-ratio decreased after 8 h of drug administration also in the non-responder group, but not 24 h after administration.http://www.sciencedirect.com/science/article/pii/S235234092200138XPhenylketonuriaPhenylalanine-to-tyrosine ratioTetrahydrobiopterinBH4-responsivenessHyperphenylalaninemiaTandem mass spectrometry |
| spellingShingle | Barbka Repic Lampret Mojca Zerjav Tansek Blaz Groselj Jaka Sikonja Tadej Battelino Urh Groselj Data on phenylalanine-to-tyrosine ratios in assessment of tetrahydrobiopterin (BH4)-responsiveness in patients with hyperphenylalaninemia Data in Brief Phenylketonuria Phenylalanine-to-tyrosine ratio Tetrahydrobiopterin BH4-responsiveness Hyperphenylalaninemia Tandem mass spectrometry |
| title | Data on phenylalanine-to-tyrosine ratios in assessment of tetrahydrobiopterin (BH4)-responsiveness in patients with hyperphenylalaninemia |
| title_full | Data on phenylalanine-to-tyrosine ratios in assessment of tetrahydrobiopterin (BH4)-responsiveness in patients with hyperphenylalaninemia |
| title_fullStr | Data on phenylalanine-to-tyrosine ratios in assessment of tetrahydrobiopterin (BH4)-responsiveness in patients with hyperphenylalaninemia |
| title_full_unstemmed | Data on phenylalanine-to-tyrosine ratios in assessment of tetrahydrobiopterin (BH4)-responsiveness in patients with hyperphenylalaninemia |
| title_short | Data on phenylalanine-to-tyrosine ratios in assessment of tetrahydrobiopterin (BH4)-responsiveness in patients with hyperphenylalaninemia |
| title_sort | data on phenylalanine to tyrosine ratios in assessment of tetrahydrobiopterin bh4 responsiveness in patients with hyperphenylalaninemia |
| topic | Phenylketonuria Phenylalanine-to-tyrosine ratio Tetrahydrobiopterin BH4-responsiveness Hyperphenylalaninemia Tandem mass spectrometry |
| url | http://www.sciencedirect.com/science/article/pii/S235234092200138X |
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