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Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis

Faculty of Health Sciences, Department of Nutrition and Dietetics, Hacettepe University, 06080 Ankara, Turkey
Department of Dietetics, Birmingham Children’s Hospital, Birmingham B4 6NH, UK
Center for Health Technology and Services Research (CINTESIS), 4200-450 Porto, Portugal
Centro de Genética Médica Dr Jacinto de Magalhães, Centro Hospitalar Universitário do Porto, 4099-028 Porto, Portugal
Centro de Referência na área das Doenças Hereditárias do Metabolismo, Centro Hospitalar Universitário do Porto—CHP EPE, 4099-001 Porto, Portugal
Beatrix Children’s Hospital, University of Groningen, University Medical Center, 9700 RB Groningen, The Netherlands
Department of PKU, Kennedy Centre, 2600 Glostrup, Denmark
Enfermedades Metabolicas Servicio de Pediatria Hospital Ramon y Cajal, 28034 Madrid, Spain
Department of Metabolism and Nutrition, Dr. von Hauner Children’s Hospital, University of Munich, 80337 Munich, Germany
Faculty of Medicine, Department of Biostatistics, Hacettepe University, 06080 Ankara, Turkey
Author to whom correspondence should be addressed.
Received: 7 August 2019 / Revised: 19 August 2019 / Accepted: 20 August 2019 / Published: 3 September 2019
There is an ongoing debate regarding the impact of phenylketonuria (PKU) and its treatment on growth. To date, evidence from studies is inconsistent, and data on the whole developmental period is limited. The primary aim of this systematic review was to investigate the effects of a phenylalanine (Phe)-restricted diet on long-term growth in patients with PKU. Four electronic databases were searched for articles published until September 2018. A total of 887 results were found, but only 13 articles met eligibility criteria. Only three studies had an adequate methodology for meta-analysis. Although the results indicate normal growth at birth and during infancy, children with PKU were significantly shorter and had lower weight for age than reference populations during the first four years of life. Impaired linear growth was observed until the end of adolescence in PKU. In contrast, growth impairment was not reported in patients with mild hyperphenylalaninemia, not requiring dietary restriction. Current evidence indicates that even with advances in dietary treatments, “optimal” growth outcomes are not attained in PKU. The majority of studies include children born before 1990s, so further research is needed to show the effects of recent dietary practices on growth in PKU. View Full-Text
Keywords: phenylketonuria; hyperphenylalaninemia; growth; anthropometrics; weight; height; z-scores phenylketonuria; hyperphenylalaninemia; growth; anthropometrics; weight; height; z-scores
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MDPI and ACS Style

Ilgaz, F.; Pinto, A.; Gökmen-Özel, H.; Rocha, J.C.; van Dam, E.; Ahring, K.; Bélanger-Quintana, A.; Dokoupil, K.; Karabulut, E.; MacDonald, A. Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis. Nutrients 2019, 11, 2070.

AMA Style

Ilgaz F, Pinto A, Gökmen-Özel H, Rocha JC, van Dam E, Ahring K, Bélanger-Quintana A, Dokoupil K, Karabulut E, MacDonald A. Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis. Nutrients. 2019; 11(9):2070.

Chicago/Turabian Style

Ilgaz, Fatma, Alex Pinto, Hülya Gökmen-Özel, Julio C. Rocha, Esther van Dam, Kirsten Ahring, Amaya Bélanger-Quintana, Katharina Dokoupil, Erdem Karabulut, and Anita MacDonald. 2019. "Long-Term Growth in Phenylketonuria: A Systematic Review and Meta-Analysis" Nutrients 11, no. 9: 2070.

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