Evaluation of trace element and mineral status and related to levels of amino acid in children with phenylketonuria |
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| Institution: | 1. Division of Metabolism, BioCruces Health Research Institute, CIBER de Enfermedades Raras (CIBERER), Plaza de Cruces 12, 48903, Barakaldo, Spain;2. Metabolic Disorders, Dietetics and Nutrition Unit, Virgen del Rocío University Hospital, Manuel Siurot Avenue s/n, 41013, Sevilla, Spain;3. Unit of Diagnosis and Treatment of Congenital Metabolic Diseases, Neonatology Service, Department of Pediatrics. Hospital Clinico Universitario de Santiago de Compostela, CIBER de Enfermedades Raras (CIBERER), IDIS, Spain;4. Nutrition and Metabolopathologies Unit, La Fe University Hospital, Bulevar Sur s/n, 46026, Valencia, Spain;5. Gastroenterology, Hepatology and Child Nutrition Unit, Carlos Haya University Hospital, Avda. Arroyo de los Ángeles s/n, 29011, Málaga, Spain;6. Paediatrics Unit, Río Hortega University Hospital, Calle Dulzaina 2, 47012, Valladolid, Spain;7. Gastroenterology Unit, Virgen de la Arrixaca University Hospital, Ctra. Madrid-Cartagena s/n, El Palmar, 30120, Murcia, Spain;8. Metabolic Pathologies Unit, Miguel Servet University Hospital, Paseo de Isabel La Católica 1-3, 50009, Zaragoza, Spain;9. Nephrology and Metabolism Unit, Marqués de Valdecilla University Hospital, Avda. Valdecilla 25, 39008, Santander, Spain;10. Paediatrics Unit, Nuestra Señora de la Candelaria University Hospital, Carretera del Rosario 145, 38010, Santa Cruz de Tenerife, Spain;11. Metabolic Pathologies and Neuropaediatrics Unit, Son Espases University Hospital, Carretera de Valldemossa 79, 07120, Palma de Mallorca, Spain;12. Paediatric Gastroenterology, Hepatology and Nutrition Unit, Mother and Child Hospital Complex, Avda. Marítima del Sur s/n, 35016, Las Palmas de Gran Canaria, Spain;13. Metabolic Pathologies Unit, Maternal and Child Hospital, Calle de la Violeta 1, 06010, Badajoz, Spain;14. Gastroenterology and Paediatric Nutrition Unit, Virgen del Camino Hospital, C/ De Irunlarrea 4, 31008, Pamplona, Spain;1. University of Groningen, University Medical Center Groningen, Division of Metabolic Diseases, PO box 30.001, 9700 RB Groningen, The Netherlands;2. Radboud University Medical Center Nijmegen, Department of Gastroenterology and Hepatology-Dietetics, PO box 9101, 6500 HB Nijmegen, The Netherlands;3. Radboud University Medical Center Nijmegen, Department of Pediatrics, PO box 9101, 6500 HB Nijmegen, The Netherlands;4. Radboud University Medical Center, Department of Internal Medicine, PO box 9101, 6500 HB Nijmegen, The Netherlands;1. The Children''s Hospital, Birmingham, UK;2. Center for PKU, The Kennedy Centre, University Hospital, Glostrup, Copenhagen, Denmark;3. Centro de Genética Médica Doutor Jacinto de Magalhães, CHP EPE, Porto, Portugal;4. Unit for Multidisciplinary Research in Biomedicine, Abel Salazar Institute of Biomedical Sciences, University of Porto-UMIB/ICBAS/UP, Porto, Portugal;5. Hospital Ramon y Cajal Madrid, Spain;6. Dietmar-Hopp Metabolic Center, University Children''s Hospital, Heidelberg, Germany;7. Division of Inherited Metabolic Diseases, University Hospital, Padua, Italy;8. Hospital for Sick Children, Great Ormond Street, London, UK;9. Department of Inherited Metabolic Disorders, Hacettepe University, Ankara, Turkey;10. Department of Metabolism and Nutrition, Dr. von Hauner Children''s Hospital, University of Munich, Munich, Germany;11. Hôpital d''enfants Brabois, Vandoeuvre les Nancy, France;12. Department of Pediatrics, Endocrinology, Diabetology, Metabolic Diseases and Cardiology, Pomeranian Medical University, Szczecin, Poland;13. Department of Nutrition and Dietetics, Hacettepe University, Ankara, Turkey;14. Dr. von Hauner Children''s Hospital, University of Munich, Munich, Germany;15. Department of Paediatrics and Haematology, Pomeranian Medical University, Szczecin, Poland;p. CHRU de Tours, service de medicine interne, Université François Rabelais, Tours, France;q. Depart Ped, San Paolo Hosp Univ Milan, Milan, Italy;r. University Children''s Hospital, University Medical Center Hamburg Eppendorf, Hamburg, Germany;s. Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium;t. Faculdade de Ciências da Saúde, Universidade Fernando Pessoa, Porto, Portugal;u. Center for Health Technology and Services Research (CINTESIS), Portugal;v. Division of Inborn Metabolic Diseases, University Children''s Hospital, Heidelberg, Germany;w. Section of Metabolic Diseases, University of Groningen, University Medical Center Groningen, Groningen, Netherlands |
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| Abstract: | The aim of the present study was to examine trace elements (Zn, Cu, Mn, Se, Fe, Co, Cr, Ni, Cd, Pb), minerals (Ca, Mg, K), amino acids status in children with phenylketonuria and also whether they were correlated with each other in phenylketonuric patients.It has been found out that the HPA group was significantly lower than the control group with regards to Zn, Se, K, Ca, Mg and Zn/Cr levels (p < 0.001, p < 0.01, p < 0.001, p < 0.01, p < 0.01 and p < 0.001 respectively). In the patients with HPA, significantly strong positive correlations were observed between magnesium and calcium (r = 0.791; p = 0.001), also, indicates negative significant correlation between the concentrations of magnesium and phenylalanine (r = ?0.591; p = 0.026).The results of this study showed that, in the HPA group, phenylalanine-Mg relationship found, the presence of disease will in the evaluation of phenylalanine and other amino acids, together with the value of magnesium is required to consider. |
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| Keywords: | Phenylketonuria Trace element Minerals Amino acids |
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