Phenylketonuric diet negatively impacts on butyrate production

Study information

Reviewed Marked as Reviewed by Atrayees on 2023-8-18

study design

case-control

Citation

PMID PubMed identifier for scientific articles.

29502926

DOI Digital object identifier for electronic documents.

10.1016/j.numecd.2018.01.004

URI

Authors

Verduci E, Moretti F, Bassanini G, Banderali G, Rovelli V, Casiraghi MC, Morace G, Borgo F, Borghi E

Journal

Nutrition, metabolism, and cardiovascular diseases : NMCD

Year

2018

Keywords:

Butyrate, Faecalibacterium prausnitzii, Phenylketonuria, Short chain fatty acids

BACKGROUND AND AIMS: Phenylalanine (Phe) restricted diet, combined with Phe-free l-amino acid supplementation, is the mainstay of treatment for phenylketonuria (PKU). Being the diet a key factor modulating gut microbiota composition, the aim of the present paper was to compare dietary intakes, gut microbiota biodiversity and short chain fatty acids (SCFAs) production in children with PKU, on low-Phe diet, and in children with mild hyperphenylalaninemia (MHP), on unrestricted diet. METHODS AND RESULTS: We enrolled 21 PKU and 21 MHP children matched for gender, age and body mass index z-score. Dietary intakes, including glycemic index (GI) and glycemic load (GL), and fecal microbiota analyses, by means of denaturing gradient gel electrophoresis (DGGE) and Real-time PCR were assessed. Fecal SCFAs were quantified by gas chromatographic analysis. RESULTS: We observed an increased carbohydrate (% of total energy), fiber and vegetables intakes (g/day) in PKU compared with MHP children (p = 0.047), as well a higher daily GI and GL (maximum p < 0.001). Compared with MHP, PKU showed a lower degree of microbial diversity and a decrease in fecal butyrate content (p = 0.02). Accordingly, two of the most abundant butyrate-producing genera, Faecalibacterium spp. and Roseburia spp., were found significantly depleted in PKU children (p = 0.02 and p = 0.03, respectively). CONCLUSION: The low-Phe diet, characterized by a higher carbohydrate intake, increases GI and GL, resulting in a different quality of substrates for microbial fermentation. Further analyses, thoroughly evaluating microbial species altered by PKU diet are needed to better investigate gut microbiota in PKU children and to eventually pave the way for pre/probiotic supplementations.

Experiment 1

Edit History Delete

Flag for review

Your review change was submittedDuplicate this Experiment Add a new Signature

Reviewed Marked as Reviewed by Atrayees on 2023-8-18

Curated date: 2021/01/10

Curator: WikiWorks

Revision editor(s): WikiWorks, Victoria

Subjects

Location of subjects: Italy

Host species Species from which microbiome was sampled. Contact us to have more species added.: Homo sapiens

Body site Anatomical site where microbial samples were extracted from according to the Uber Anatomy Ontology: Feces Cow dung,Cow pat,Droppings,Dung,Excrement,Excreta,Faeces,Fecal material,Fecal matter,Fewmet,Frass,Guano,Matières fécales@fr,Merde@fr,Ordure,Partie de la merde@fr,Piece of shit,Porción de mierda@es,Portion of dung,Portion of excrement,Portion of faeces,Portion of fecal material,Portion of fecal matter,Portion of feces,Portion of guano,Portion of scat,Portionem cacas,Scat,Spoor,Spraint,Stool,Teil der fäkalien@de,Feces,feces

Condition The experimental condition / phenotype studied according to the Experimental Factor Ontology: Phenylketonuria Folling disease,Folling's disease,Folling's syndrome,Følling's disease,HPA, non-PKU mild,hyperphenylalaninemia, non-PKU mild,imbecilitus phenylpyruvica,oligophrenia Phenylpyruvica,oligophrenia phenylpyruvica,PAH deficiency,Pah deficiency,pah deficiency,Phenylalanine hydroxylase deficiency,phenylalanine hydroxylase deficiency,phenylalaninemia,phenylketonuria,phenylketonuria, maternal,phenylpyruvic oligophrenia,PKU,Phenylketonuria

Group 0 name Corresponds to the control (unexposed) group for case-control studies: mild hyperphenylalaninemic with unrestricted diet

Group 1 name Corresponds to the case (exposed) group for case-control studies: phenylketonuric with Phe-restricted diet

Group 1 definition Diagnostic criteria applied to define the specific condition / phenotype represented in the case (exposed) group: phenylketonuric patients with restricted (phenylalanine-free) diets

Group 0 sample size Number of subjects in the control (unexposed) group: 21

Group 1 sample size Number of subjects in the case (exposed) group: 21

Antibiotics exclusion Number of days without antibiotics usage (if applicable) and other antibiotics-related criteria used to exclude participants (if any): 3 months

Lab analysis

Sequencing type: 16S

16S variable region One or more hypervariable region(s) of the bacterial 16S gene: V2-V3

Sequencing platform Manufacturer and experimental platform used for quantifying microbial abundance: RT-qPCR

Statistical Analysis

Data transformation Data transformation applied to microbial abundance measurements prior to differential abundance testing (if any).: relative abundances

Statistical test: Mann-Whitney (Wilcoxon)

Significance threshold p-value or FDR threshold used for differential abundance testing (if any): 0.05

MHT correction Have statistical tests be corrected for multiple hypothesis testing (MHT)?: No

Matched on Factors on which subjects have been matched on in a case-control study: age, sex

Signature 1

Edit History Delete

Flag for review

Your review change was submitted

Reviewed Marked as Reviewed by Atrayees on 2023-8-18

Curated date: 2021/01/10

Curator: Aboud Ezzeddine

Revision editor(s): WikiWorks

Source: Figure 2

Description: Microbial quantiﬁcation, in phenylketonuric (PKU) and mild hyperphenylalaninemia (MHP) children, of Faecalibacterium prausnitzii, and Roseburia spp

Abundance in Group 1: decreased abundance in phenylketonuric with Phe-restricted diet

NCBI	Quality Control	Links
Faecalibacterium prausnitzii
Roseburia
Lactobacillus

Revision editor(s): WikiWorks