PMID- 34946200
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20211229
IS  - 2076-2607 (Print)
IS  - 2076-2607 (Electronic)
IS  - 2076-2607 (Linking)
VI  - 9
IP  - 12
DP  - 2021 Dec 16
TI  - Assessing Gut Microbiota in an Infant with Congenital Propionic Acidemia before 
      and after Probiotic Supplementation.
LID - 10.3390/microorganisms9122599 [doi]
LID - 2599
AB  - Propionic Acidemia (PA) is a rare inherited metabolic disorder caused by the 
      enzymatic block of propionyl-CoA carboxylase with the consequent accumulation of 
      propionic acid, which is toxic for the brain and cardiac cells. Since a 
      considerable amount of propionate is produced by intestinal bacteria, interest 
      arose in the attempt to reduce propionate-producing bacteria through a monthly 
      antibiotic treatment of metronidazole. In the present study, we investigated the 
      gut microbiota structure of an infant diagnosed at 4 days of life through 
      Expanded Newborn Screening (NBS) and treated the child following international 
      guidelines with a special low-protein diet, specific medications and strict 
      biochemical monitoring. Microbiota composition was assessed during the first 
      month of life, and the presence of Bacteroides fragilis, known to be associated 
      with propionate production, was effectively decreased by metronidazole treatment. 
      After five antibiotic therapy cycles, at 4 months of age, the infant was 
      supplemented with a daily mixture of three bifidobacterial strains, known not to 
      be propionate producers. The supplementation increased the population of 
      bifidobacteria, with Bifidobacterium breve as the dominating species; 
      Ruminococcus gnavus, an acetate and formate producer, was also identified. 
      Metabarcoding analysis, compared with low coverage whole metagenome sequencing, 
      proved to capture all the microbial biodiversity and could be the elected tool 
      for fast and cost-effective monitoring protocols to be implemented in the follow 
      up of rare metabolic disorders such as PA. Data obtained could be a possible 
      starting point to set up tailored microbiota modification treatment studies in 
      the attempt to improve the quality of life of people affected by propionic 
      acidemia.
FAU - Bordugo, Andrea
AU  - Bordugo A
AUID- ORCID: 0000-0003-1927-8187
AD  - Inherited Metabolic Disease Unit, Pediatric Clinic C, Azienda Ospedaliera 
      Universita Integrata, Piazzale Stefani 1, 37126 Verona, Italy.
FAU - Salvetti, Elisa
AU  - Salvetti E
AD  - Department of Biotechnology, University of Verona, Strada le Grazie 15, 37134 
      Verona, Italy.
FAU - Rodella, Giulia
AU  - Rodella G
AD  - Inherited Metabolic Disease Unit, Pediatric Clinic C, Azienda Ospedaliera 
      Universita Integrata, Piazzale Stefani 1, 37126 Verona, Italy.
AD  - Department of Surgery, Dentistry, Paediatrics and Gynaecology, University of 
      Verona, Piazzale Stefani 1, 37126 Verona, Italy.
FAU - Piazza, Michele
AU  - Piazza M
AUID- ORCID: 0000-0001-7746-8267
AD  - Department of Surgery, Dentistry, Paediatrics and Gynaecology, University of 
      Verona, Piazzale Stefani 1, 37126 Verona, Italy.
FAU - Dianin, Alice
AU  - Dianin A
AD  - Inherited Metabolic Disease Unit, Pediatric Clinic C, Azienda Ospedaliera 
      Universita Integrata, Piazzale Stefani 1, 37126 Verona, Italy.
FAU - Amoruso, Angela
AU  - Amoruso A
AD  - Probiotical Research Srl, Via Enrico Mattei 3, 28100 Novara, Italy.
FAU - Piacentini, Giorgio
AU  - Piacentini G
AD  - Department of Surgery, Dentistry, Paediatrics and Gynaecology, University of 
      Verona, Piazzale Stefani 1, 37126 Verona, Italy.
FAU - Pane, Marco
AU  - Pane M
AD  - Probiotical Research Srl, Via Enrico Mattei 3, 28100 Novara, Italy.
FAU - Torriani, Sandra
AU  - Torriani S
AUID- ORCID: 0000-0002-2565-7557
AD  - Department of Biotechnology, University of Verona, Strada le Grazie 15, 37134 
      Verona, Italy.
FAU - Vitulo, Nicola
AU  - Vitulo N
AUID- ORCID: 0000-0002-9571-0747
AD  - Department of Biotechnology, University of Verona, Strada le Grazie 15, 37134 
      Verona, Italy.
FAU - Felis, Giovanna E
AU  - Felis GE
AD  - Department of Biotechnology, University of Verona, Strada le Grazie 15, 37134 
      Verona, Italy.
LA  - eng
PT  - Case Reports
DEP - 20211216
PL  - Switzerland
TA  - Microorganisms
JT  - Microorganisms
JID - 101625893
PMC - PMC8703847
OTO - NOTNLM
OT  - Bacteroides fragilis
OT  - Bifidobacterium breve
OT  - Ruminococcus gnavus
OT  - VOC
OT  - bifidobacteria
OT  - diet
OT  - electronic nose
OT  - genetic
OT  - gut microbiota
OT  - inherited metabolic diseases
OT  - metabarcoding
OT  - metagenomics
OT  - metronidazole
OT  - organic acidurias
OT  - probiotic supplementation
OT  - propionic acid
OT  - propionic acidemia
COIS- Marco Pane and Angela Amoruso are employees of Probiotical Research Srl (Novara, 
      Italy).
EDAT- 2021/12/25 06:00
MHDA- 2021/12/25 06:01
PMCR- 2021/12/16
CRDT- 2021/12/24 01:10
PHST- 2021/10/29 00:00 [received]
PHST- 2021/11/25 00:00 [revised]
PHST- 2021/12/13 00:00 [accepted]
PHST- 2021/12/24 01:10 [entrez]
PHST- 2021/12/25 06:00 [pubmed]
PHST- 2021/12/25 06:01 [medline]
PHST- 2021/12/16 00:00 [pmc-release]
AID - microorganisms9122599 [pii]
AID - microorganisms-09-02599 [pii]
AID - 10.3390/microorganisms9122599 [doi]
PST - epublish
SO  - Microorganisms. 2021 Dec 16;9(12):2599. doi: 10.3390/microorganisms9122599.