(Q37990774)

26 August 2017

Tetrahydrobiopterin (BH4) in PKU: effect on dietary treatment, metabolic control, and quality of life (English)

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26 August 2017

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ChEBI release 2020-05-28

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B Ziesch

1

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26 August 2017

J Weigel

2

1 reference

26 August 2017

A Thiele

3

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26 August 2017

U Mütze

4

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26 August 2017

C Rohde

5

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26 August 2017

U Ceglarek

6

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26 August 2017

J Thiery

7

1 reference

26 August 2017

W Kiess

8

1 reference

26 August 2017

S Beblo

9

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26 August 2017

6 March 2012

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Journal of Inherited Metabolic Disease

26 August 2017

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26 August 2017

35

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26 August 2017

6

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26 August 2017

983-992

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https://scigraph.springernature.com/pub.10.1007/s10545-012-9458-1

26 August 2017

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cites work

High frequency of tetrahydrobiopterin-responsiveness among hyperphenylalaninemias: a study of 1,919 patients observed from 1988 to 2002.

1 reference

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Quality of life in noncompliant adults with phenylketonuria after resumption of the diet

21 January 2018

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Living with phenylketonuria: perspectives of patients and their families

21 January 2018

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The course of life and quality of life of early and continuously treated Dutch patients with phenylketonuria.

21 January 2018

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Psychiatric symptoms and disorders in phenylketonuria.

21 January 2018

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Neuropsychologic functions of early treated patients with phenylketonuria, on and off diet: results of a cross-national and cross-sectional study.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Effect of BH(4) supplementation on phenylalanine tolerance

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Sapropterin therapy increases stability of blood phenylalanine levels in patients with BH4-responsive phenylketonuria (PKU).

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Validation of the phenylalanine/tyrosine ratio determined by tandem mass spectrometry: sensitive newborn screening for phenylketonuria

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Incidence of BH4-responsiveness in phenylalanine-hydroxylase-deficient Italian patients

21 January 2018

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Hidden consequences of success in pediatrics: parental health-related quality of life--results from the Care Project.

21 January 2018

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A predictive model of Health Related Quality of life of parents of chronically ill children: the importance of care-dependency of their child and their support system

21 January 2018

1 reference

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Long-term treatment with tetrahydrobiopterin increases phenylalanine tolerance in children with severe phenotype of phenylketonuria

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Quality of life and psychologic adjustment in children and adolescents with early treated phenylketonuria can be normal

21 January 2018

1 reference

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Tetrahydrobiopterin responsiveness in phenylketonuria differs between patients with the same genotype

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Diet and compliance in phenylketonuria

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

The reality of dietary compliance in the management of phenylketonuria

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Tetrahydrobiopterin as an alternative treatment for mild phenylketonuria

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Transition of young adults with phenylketonuria from pediatric to adult care

21 January 2018

1 reference

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Mechanisms underlying responsiveness to tetrahydrobiopterin in mild phenylketonuria mutations

21 January 2018

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Neurological outcome in adult patients with early-treated phenylketonuria.

21 January 2018

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21 January 2018

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Long-term treatment and diagnosis of tetrahydrobiopterin-responsive hyperphenylalaninemia with a mutant phenylalanine hydroxylase gene

21 January 2018

1 reference

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Evaluation of quality of life and description of the sociodemographic state in adolescent and young adult patients with phenylketonuria (PKU).

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Behaviour and school achievement in patients with early and continuously treated phenylketonuria.

21 January 2018

1 reference

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Parenting a child with phenylketonuria or galactosemia: implications for health-related quality of life.

21 January 2018

1 reference

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Neurological deterioration in young adults with phenylketonuria

21 January 2018

1 reference

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Efficacy of sapropterin dihydrochloride in increasing phenylalanine tolerance in children with phenylketonuria: a phase III, randomized, double-blind, placebo-controlled study

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Psychological and social findings in adolescents with phenylketonuria

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Molecular genetics of tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Parent Functioning in Families of Preadolescents With Spina Bifida: Longitudinal Implications for Child Adjustment

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FS10545-012-9458-1

Assessment of tetrahydrobiopterin (BH4) responsiveness in phenylketonuria

21 January 2018

1 reference

12 December 2020

Treatment products and approaches for phenylketonuria: improved palatability and flexibility demonstrate safety, efficacy and acceptance in US clinical trials

1 reference

12 December 2020

The effect of blood phenylalanine concentration on Kuvan™ response in phenylketonuria

1 reference

12 December 2020

Tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency

1 reference

12 December 2020

PKU in adolescents: rationale and psychosocial factors in diet continuation.

1 reference

12 December 2020

Effects of pediatric chronic physical disorders on child and family adjustment

1 reference

12 December 2020

Safety and efficacy of 22 weeks of treatment with sapropterin dihydrochloride in patients with phenylketonuria

1 reference

12 December 2020

Sapropterin dihydrochloride (Kuvan/phenoptin), an orally active synthetic form of BH4 for the treatment of phenylketonuria

1 reference

12 December 2020

Coping with PKU: results of narrative interviews with parents

1 reference

12 December 2020

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

1 reference

12 December 2020

10.1007/S10545-012-9458-1

Identifiers

DOI

1 reference

26 August 2017

1 reference