Literature DB >> 23946023

Caring for children with phenylketonuria.

Linda Casey1.   

Abstract

OBJECTIVE: To provide an overview of the diagnosis and management of phenylketonuria (PKU) in childhood with an emphasis on aspects relevant to family physicians providing ongoing care. SOURCES OF INFORMATION: The author's experience as the clinic physician in a regional pediatric PKU clinic is supplemented with references providing evidence for key points. MAIN MESSAGE: While metabolic clinics typically provide guidance regarding the specific management of PKU, the family doctor has an essential role in providing ongoing medical care.
CONCLUSION: Children and families have much to gain from strong relationships with family doctors, and family doctors can confidently provide care with awareness of the very few potential special needs of patients with PKU.

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Mesh:

Year:  2013        PMID: 23946023      PMCID: PMC3743692     

Source DB:  PubMed          Journal:  Can Fam Physician        ISSN: 0008-350X            Impact factor:   3.275


  7 in total

Review 1.  Phenylketonuria (PKU): screening and management.

Authors: 
Journal:  NIH Consens Statement       Date:  2000 Oct 16-18

2.  Neuropsychological outcome of subjects participating in the PKU adult collaborative study: a preliminary review.

Authors:  V L Brumm; C Azen; R A Moats; A M Stern; C Broomand; M D Nelson; R Koch
Journal:  J Inherit Metab Dis       Date:  2004       Impact factor: 4.982

3.  School performance in early and continuously treated phenylketonuria.

Authors:  Rosa Gassió; Eugenia Fusté; Anna López-Sala; Rafael Artuch; María Antonia Vilaseca; Jaume Campistol
Journal:  Pediatr Neurol       Date:  2005-10       Impact factor: 3.372

Review 4.  ADHD, learning, and academic performance in phenylketonuria.

Authors:  Kevin M Antshel
Journal:  Mol Genet Metab       Date:  2010       Impact factor: 4.797

Review 5.  The role of intelligence in phenylketonuria: a review of research and management.

Authors:  V L Brumm; M L Grant
Journal:  Mol Genet Metab       Date:  2010       Impact factor: 4.797

6.  Do adult patients with phenylketonuria improve their quality of life after introduction/resumption of a phenylalanine-restricted diet?

Authors:  R Gassió; J Campistol; M A Vilaseca; N Lambruschini; F J Cambra; E Fusté
Journal:  Acta Paediatr       Date:  2003-12       Impact factor: 2.299

Review 7.  Large neutral amino acids in the treatment of PKU: from theory to practice.

Authors:  Francjan J van Spronsen; Martijn J de Groot; Marieke Hoeksma; Dirk-Jan Reijngoud; Margreet van Rijn
Journal:  J Inherit Metab Dis       Date:  2010-10-26       Impact factor: 4.982

  7 in total
  7 in total

Review 1.  Transdisciplinary approaches enhance the production of translational knowledge.

Authors:  Timothy H Ciesielski; Melinda C Aldrich; Carmen J Marsit; Robert A Hiatt; Scott M Williams
Journal:  Transl Res       Date:  2016-11-10       Impact factor: 7.012

2.  Preventing brain disorders: a framework for action.

Authors:  Sasha Rudenstine; Sandro Galea
Journal:  Soc Psychiatry Psychiatr Epidemiol       Date:  2015-01-17       Impact factor: 4.328

3.  Protective effect of L-carnitine on Phenylalanine-induced DNA damage.

Authors:  Marion Deon; Sharon S Landgraf; Jessica F Lamberty; Dinara J Moura; Jenifer Saffi; Moacir Wajner; Carmen R Vargas
Journal:  Metab Brain Dis       Date:  2015-01-20       Impact factor: 3.584

4.  In Vivo Metabolic Responses to Different Formulations of Amino Acid Mixtures for the Treatment of Phenylketonuria (PKU).

Authors:  Nadia Giarratana; Luciana Giardino; Andrea Bighinati; Giorgio Reiner; Júlio César Rocha
Journal:  Int J Mol Sci       Date:  2022-02-17       Impact factor: 5.923

5.  Pain points in parents' interactions with newborn screening systems: a qualitative study.

Authors:  Mike Conway; Truc Thuy Vuong; Kim Hart; Andreas Rohrwasser; Karen Eilbeck
Journal:  BMC Pediatr       Date:  2022-03-31       Impact factor: 2.125

6.  L-carnitine protects DNA oxidative damage induced by phenylalanine and its keto acid derivatives in neural cells: a possible pathomechanism and adjuvant therapy for brain injury in phenylketonuria.

Authors:  Jéssica Lamberty Faverzani; Aline Steinmetz; Marion Deon; Desirèe Padilha Marchetti; Gilian Guerreiro; Angela Sitta; Daniella de Moura Coelho; Franciele Fatima Lopes; Leopoldo Vinicius Martins Nascimento; Luiza Steffens; Jeferson Gustavo Henn; Matheus Bernardes Ferro; Verônica Bidinotto Brito; Moacir Wajner; Dinara Jaqueline Moura; Carmen Regla Vargas
Journal:  Metab Brain Dis       Date:  2021-07-03       Impact factor: 3.584

7.  Health economic burden of patients with phenylketonuria (PKU) - A retrospective study of German health insurance claims data.

Authors:  Friedrich Trefz; Ania C Muntau; Kim M Schneider; Julia Altevers; Christian Jacob; Sebastian Braun; Wolfgang Greiner; Ashok Jha; Mohit Jain; Ignacio Alvarez; Paul Lane; Claudia Zeiss; Frank Rutsch
Journal:  Mol Genet Metab Rep       Date:  2021-05-13
  7 in total

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