Fatty liver disease in children (MAFLD/PeFLD Type 2): unique classification considerations and challenges

In children, fatty liver disease is a group of disorders that often overlaps with inherited metabolic disorders (IMDs), which requires prompt diagnosis and specific management. Metabolic dysfunction–associated fatty liver disease (MAFLD) or, formerly, non-alcoholic fatty liver disease (NAFLD) is the...

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Autores principales: Hegarty, Robert, Kyrana, Eirini, Fitzpatrick, Emer, Dhawan, Anil
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10034351/
https://www.ncbi.nlm.nih.gov/pubmed/36968656
http://dx.doi.org/10.1177/20420188231160388
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author Hegarty, Robert
Kyrana, Eirini
Fitzpatrick, Emer
Dhawan, Anil
author_facet Hegarty, Robert
Kyrana, Eirini
Fitzpatrick, Emer
Dhawan, Anil
author_sort Hegarty, Robert
collection PubMed
description In children, fatty liver disease is a group of disorders that often overlaps with inherited metabolic disorders (IMDs), which requires prompt diagnosis and specific management. Metabolic dysfunction–associated fatty liver disease (MAFLD) or, formerly, non-alcoholic fatty liver disease (NAFLD) is the hepatic component of a multisystemic disease that requires a positive criteria in metabolic dysfunction for diagnosis. However, in children, the diagnosis of MAFLD is one of the exclusions of an IMD [paediatric fatty liver disease (PeFLD) type 1] including the possibility that an IMD can be identified in the future following investigations that may be negative at the time. Therefore, while children with fatty liver with metabolic dysfunction could be classified as MAFLD (PeFLD type 2) and managed that way, those who do not fulfil the criteria for metabolic dysfunction should be considered separately bearing in mind the possibility of identifying a yet undiagnosed IMD (PeFLD type 3). This concept is ever more important in a world where MAFLD is the most common cause of liver disease in children and adolescents in whom about 7% are affected. The disease is only partially understood, and awareness is still lacking outside hepatology and gastroenterology. Despite its increasing pervasiveness, the management is far from a one-size-fits-all. Increasing complexities around the genetic, epigenetic, non-invasive modalities of assessment, psychosocial impacts, therapeutics, and natural history of the disease have meant that an individualised approach is required. This is where the challenge lies so that children with fatty liver are considered on their own merits. The purpose of this review is to give a clinical perspective of fatty liver disease in children with relevance to metabolic dysfunction.
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spelling pubmed-100343512023-03-24 Fatty liver disease in children (MAFLD/PeFLD Type 2): unique classification considerations and challenges Hegarty, Robert Kyrana, Eirini Fitzpatrick, Emer Dhawan, Anil Ther Adv Endocrinol Metab New Insights in MAFLD In children, fatty liver disease is a group of disorders that often overlaps with inherited metabolic disorders (IMDs), which requires prompt diagnosis and specific management. Metabolic dysfunction–associated fatty liver disease (MAFLD) or, formerly, non-alcoholic fatty liver disease (NAFLD) is the hepatic component of a multisystemic disease that requires a positive criteria in metabolic dysfunction for diagnosis. However, in children, the diagnosis of MAFLD is one of the exclusions of an IMD [paediatric fatty liver disease (PeFLD) type 1] including the possibility that an IMD can be identified in the future following investigations that may be negative at the time. Therefore, while children with fatty liver with metabolic dysfunction could be classified as MAFLD (PeFLD type 2) and managed that way, those who do not fulfil the criteria for metabolic dysfunction should be considered separately bearing in mind the possibility of identifying a yet undiagnosed IMD (PeFLD type 3). This concept is ever more important in a world where MAFLD is the most common cause of liver disease in children and adolescents in whom about 7% are affected. The disease is only partially understood, and awareness is still lacking outside hepatology and gastroenterology. Despite its increasing pervasiveness, the management is far from a one-size-fits-all. Increasing complexities around the genetic, epigenetic, non-invasive modalities of assessment, psychosocial impacts, therapeutics, and natural history of the disease have meant that an individualised approach is required. This is where the challenge lies so that children with fatty liver are considered on their own merits. The purpose of this review is to give a clinical perspective of fatty liver disease in children with relevance to metabolic dysfunction. SAGE Publications 2023-03-22 /pmc/articles/PMC10034351/ /pubmed/36968656 http://dx.doi.org/10.1177/20420188231160388 Text en © The Author(s), 2023 https://creativecommons.org/licenses/by/4.0/This article is distributed under the terms of the Creative Commons Attribution 4.0 License (https://creativecommons.org/licenses/by/4.0/) which permits any use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle New Insights in MAFLD
Hegarty, Robert
Kyrana, Eirini
Fitzpatrick, Emer
Dhawan, Anil
Fatty liver disease in children (MAFLD/PeFLD Type 2): unique classification considerations and challenges
title Fatty liver disease in children (MAFLD/PeFLD Type 2): unique classification considerations and challenges
title_full Fatty liver disease in children (MAFLD/PeFLD Type 2): unique classification considerations and challenges
title_fullStr Fatty liver disease in children (MAFLD/PeFLD Type 2): unique classification considerations and challenges
title_full_unstemmed Fatty liver disease in children (MAFLD/PeFLD Type 2): unique classification considerations and challenges
title_short Fatty liver disease in children (MAFLD/PeFLD Type 2): unique classification considerations and challenges
title_sort fatty liver disease in children (mafld/pefld type 2): unique classification considerations and challenges
topic New Insights in MAFLD
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10034351/
https://www.ncbi.nlm.nih.gov/pubmed/36968656
http://dx.doi.org/10.1177/20420188231160388
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