Evidence map›Paper›PMID 31752110›Full record

ArticleNutrients2019

The Effect of Various Doses of Phenylalanine Supplementation on Blood Phenylalanine and Tyrosine Concentrations in Tyrosinemia Type 1 Patients.

Willem G van Ginkel, Hannah E van Reemst, Nienke S Kienstra, Anne Daly, Iris L Rodenburg, Anita MacDonald, Johannes G M Burgerhof, Pim de Blaauw, Jennifer van de Krogt, Saikat Santra and 2 more

Open access · goldAbstract readMulticenter Study
In one paragraph

Article in Nutrients, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed
0.8field-weighted citation impact, top 29% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

7 citing papers in PubMed, 10 citations in OpenAlex.

  1. Review
  2. Review
  3. Article
  4. Article
  5. Article
  6. Hereditary tyrosinemia type Ⅰ: newborn screening, diagnosis and treatment.Zhejiang da xue xue bao. Yi xue ban = Journal of Zhejiang University. Medical sciences · 2021
    Article
  7. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

12 authors at 3 institutions in 2 countries.

Willem G van GinkelDepartment of Metabolic Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, 9713 GZ Groningen, The Netherlands.
Hannah E van ReemstDepartment of Metabolic Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, 9713 GZ Groningen, The Netherlands.
Nienke S KienstraDepartment of Metabolic Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, 9713 GZ Groningen, The Netherlands.
Anne DalyDepartment of Metabolic Diseases, Birmingham Children's Hospital, Birmingham B4 6NH, UK.
Iris L RodenburgDepartment of Dietetics, University Medical Center Groningen, University of Groningen, 9713 GZ Groningen, The Netherlands.
Anita MacDonaldDepartment of Metabolic Diseases, Birmingham Children's Hospital, Birmingham B4 6NH, UK.
Johannes G M BurgerhofDepartment of Epidemiology, University Medical Center Groningen, University of Groningen, 9713 GZ Groningen, The Netherlands.
Pim de BlaauwDepartment of Laboratory Medicine, University Medical Center Groningen, University of Groningen, 9713 GZ Groningen, The Netherlands.
Jennifer van de KrogtDepartment of Laboratory Medicine, University Medical Center Groningen, University of Groningen, 9713 GZ Groningen, The Netherlands.
Saikat SantraDepartment of Metabolic Diseases, Birmingham Children's Hospital, Birmingham B4 6NH, UK.
M Rebecca Heiner-FokkemaDepartment of Laboratory Medicine, University Medical Center Groningen, University of Groningen, 9713 GZ Groningen, The Netherlands.
Francjan J van SpronsenDepartment of Metabolic Diseases, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, 9713 GZ Groningen, The Netherlands.
University Medical Center Groningen · NLUniversity of Groningen · NLBirmingham Children's Hospital · GB

Funding

MetaKids 2016-066
6 · The paper itself

Abstract

Tyrosinemia type 1 (TT1) treatment with 2-(2-nitro-4-trifluormethyl-benzyl)-1,3-cyclohexanedione (NTBC) and a phenylalanine-tyrosine restricted diet is associated with low phenylalanine concentrations. Phenylalanine supplementation is prescribed without comprehensive consideration about its effect on metabolic control. We investigated the effect of phenylalanine supplementation on bloodspot phenylalanine, tyrosine, NTBC and succinylacetone. Eleven TT1 patients received 0, 20 and 40 mg/kg/day phenylalanine supplementation with the phenylalanine-tyrosine free L-amino acid supplements. Bloodspots were collected before breakfast, midday and evening meal. Differences between study periods, sample times and days within a study period were studied using (generalized) linear mixed model analyses. Twenty and 40 mg/kg/day phenylalanine supplementation prevented daytime phenylalanine decreases (

Indexed as

AdolescentAdultChildCyclohexanonesDietary SupplementsEnzyme InhibitorsFemaleHeptanoatesHumansLinear ModelsMaleNitrobenzoatesPhenylalanineTyrosineTyrosinemiasYoung AdultCyclohexanonesEnzyme InhibitorsHeptanoatesnitisinoneNitrobenzoatesPhenylalaninesuccinylacetoneTyrosinephenylalaninesupplementationtyrosinetyrosinemia type 1

Identifiers

PMID31752110
PMCPMC6893509
OpenAlexW2984857692

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.