Lymphocyte Immune Response and T Cell Differentiation in Fontan Patients with protein-losing enteropathy.
Adolescent
Animals
Autoimmunity
Case-Control Studies
Cell Differentiation
Child
Child, Preschool
Databases, Factual
Female
Fontan Procedure
/ adverse effects
Gene Expression Profiling
Gene Regulatory Networks
Heart Defects, Congenital
/ surgery
Humans
Immunophenotyping
Infant
Lymphopenia
/ diagnosis
Male
Mice
MicroRNAs
/ genetics
Phenotype
Protein-Losing Enteropathies
/ diagnosis
T-Lymphocyte Subsets
/ immunology
Transcriptome
Treatment Outcome
Young Adult
Journal
The Thoracic and cardiovascular surgeon
ISSN: 1439-1902
Titre abrégé: Thorac Cardiovasc Surg
Pays: Germany
ID NLM: 7903387
Informations de publication
Date de publication:
12 2021
12 2021
Historique:
entrez:
19
2
2021
pubmed:
20
2
2021
medline:
29
4
2021
Statut:
ppublish
Résumé
Protein-losing enteropathy (PLE) is a severe complication of the Fontan circulation. There is increasing discussion about whether lymphatic dysregulation is involved as pathomechanism of PLE. This investigation focuses on the interplay between alteration of lymphatic cells and immunologic pathway alterations. Micro-ribonucleic acid (miRNA) expression profiling was performed in 49 patients ( miRNAs pathway analysis of Fontan patients with PLE revealed 20 significantly changed networks of which four of the ten largest were associated with immunologic processes. This finding is supported by significant T cell deficiency with decreased CD4+ count ( PLE in Fontan patients is associated with severe lymphopenia, T cell deficiency, significant alterations of T cell differentiation, and increased Treg frequency reflecting an immune status of chronic inflammation and shortened protection against pathogens and autoimmunity. These cellular alterations seemed to be dysregulated by several miRNA controlled immunological pathways.
Sections du résumé
BACKGROUND
Protein-losing enteropathy (PLE) is a severe complication of the Fontan circulation. There is increasing discussion about whether lymphatic dysregulation is involved as pathomechanism of PLE. This investigation focuses on the interplay between alteration of lymphatic cells and immunologic pathway alterations.
METHODS
Micro-ribonucleic acid (miRNA) expression profiling was performed in 49 patients (
RESULTS
miRNAs pathway analysis of Fontan patients with PLE revealed 20 significantly changed networks of which four of the ten largest were associated with immunologic processes. This finding is supported by significant T cell deficiency with decreased CD4+ count (
CONCLUSION
PLE in Fontan patients is associated with severe lymphopenia, T cell deficiency, significant alterations of T cell differentiation, and increased Treg frequency reflecting an immune status of chronic inflammation and shortened protection against pathogens and autoimmunity. These cellular alterations seemed to be dysregulated by several miRNA controlled immunological pathways.
Identifiants
pubmed: 33607694
doi: 10.1055/s-0041-1723781
pmc: PMC7909601
doi:
Substances chimiques
MicroRNAs
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
e10-e20Informations de copyright
The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).
Déclaration de conflit d'intérêts
The authors have no conflicts of interest to declare.
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