Literature DB >> 25174964

Fifty years of phenylketonuria newborn screening - A great success for many, but what about the rest?

Urh Groselj1, Mojca Zerjav Tansek1, Tadej Battelino2.   

Abstract

Guthrie's landmark discovery and the subsequent implementation of the first newborn screening programs for phenylketonuria (PKU) and other inherited errors of metabolism (IEM) could be - in a 50 year retrospective - easily considered among the greatest advances in medicine. They have not just improved the quality of hundreds of thousands of lives, but also transformed our understanding and approach to PKU and IEM in general. However, according to the available albeit very scarce data, many countries and regions seem not to share the benefits of the last 50 years of development. Many of them have not yet introduced the newborn screening for PKU or face significant problems in its implementation. In addition, the issue seems to be underrated by the relevant professional forums. Action to improve the current situation should urgently be taken.
Copyright © 2014 Elsevier Inc. All rights reserved.

Entities:  

Keywords:  Guthrie test; Newborn screening; Phenylketonuria

Mesh:

Year:  2014        PMID: 25174964     DOI: 10.1016/j.ymgme.2014.07.019

Source DB:  PubMed          Journal:  Mol Genet Metab        ISSN: 1096-7192            Impact factor:   4.797


  15 in total

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Authors:  Charlotte Veyrat-Durebex; Christelle Debeissat; Hélène Blasco; Franck Patin; Hélène Henique; Patrick Emond; Catherine Antar; Valérie Gissot; Olivier Herault; François Maillot
Journal:  JIMD Rep       Date:  2017-03-15

2.  PLASMA PHENYLALANINE DETERMINATION BY QUANTITATIVE DENSITOMETRY OF THIN LAYER CHROMATOGRAMS AND BY HIGH PERFORMANCE LIQUID CHROMATOGRAPHY IN RELATION WITH MODERN MANAGEMENT OF PHENYLKETONURIA.

Authors:  C V Mihali; C M Petrescu; F C Ladaşiu-Ciolacu; I Mândruţiu; D Bechet; T Nistor; A Ardelean; Gh Benga
Journal:  Acta Endocrinol (Buchar)       Date:  2018 Oct-Dec       Impact factor: 0.877

3.  COMPARISON OF PLASMA PHENYLALANINE DETERMINATION BY DENSITOMETRY OF THIN-LAYER CHROMATOGRAMS AND BY HIGH PERFORMANCE LIQUID CHROMATOGRAPHY IN RELATION WITH THE SCREENING OF PHENYLKETONURIA.

Authors:  C V Mihali; M C Petrescu; I Mândruţiu; D Bechet; T V Nistor; V Turcuş; A Ardelean; Gh Benga
Journal:  Acta Endocrinol (Buchar)       Date:  2017 Apr-Jun       Impact factor: 0.877

4.  Very Long-Chain Acyl-CoA Dehydrogenase Deficiency: High Incidence of Detected Patients With Expanded Newborn Screening Program.

Authors:  Ziga I Remec; Urh Groselj; Ana Drole Torkar; Mojca Zerjav Tansek; Vanja Cuk; Dasa Perko; Blanka Ulaga; Neza Lipovec; Marusa Debeljak; Jernej Kovac; Tadej Battelino; Barbka Repic Lampret
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5.  Phenylketonuria screening and management in southeastern Europe - survey results from 11 countries.

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6.  Phenylketonuria screening in the Republic of Macedonia.

Authors:  Mirjana Kocova; Violeta Anastasovska
Journal:  Orphanet J Rare Dis       Date:  2016-08-05       Impact factor: 4.123

7.  Diagnostic and management practices for phenylketonuria in 19 countries of the South and Eastern European Region: survey results.

Authors:  Maria Giżewska; Anita MacDonald; Amaya Bélanger-Quintana; Alberto Burlina; Maureen Cleary; Turgay Coşkun; François Feillet; Ania C Muntau; Friedrich K Trefz; Francjan J van Spronsen; Nenad Blau
Journal:  Eur J Pediatr       Date:  2015-09-08       Impact factor: 3.183

8.  Phenylketonuria patients' and their parents' knowledge and attitudes to the daily diet - multi-centre study.

Authors:  Ewa Witalis; Bozena Mikoluc; Radoslaw Motkowski; Jolanta Sawicka-Powierza; Agnieszka Chrobot; Bozena Didycz; Agata Lange; Renata Mozrzymas; Andrzej Milanowski; Maria Nowacka; Mariola Piotrowska-Depta; Hanna Romanowska; Ewa Starostecka; Jolanta Wierzba; Magdalena Skorniewska; Barbara Iwona Wojcicka-Bartlomiejczyk; Maria Gizewska; Halina Car
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9.  Nano-biosensor based on reduced graphene oxide and gold nanoparticles, for detection of phenylketonuria-associated DNA mutation.

Authors:  Seyed Morteza Seifati; Navid Nasirizadeh; Mostafa Azimzadeh
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10.  Challenges of Implementation of the National Phenylketonuria Screening Program in Iran: A Qualitative Study.

Authors:  Alireza Heidari; Mohammad Arab; Koorosh Etemad; Behzad Damari; Mohammad Javad Kabir
Journal:  Electron Physician       Date:  2016-10-25
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