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Pain in Mucopolysaccharidoses: Analysis of the Problem and Possible Treatments

Mucopolysaccharidosis (MPS) are a group of lysosomal storage disorders that are caused by the deficiency of enzymes involving in the catabolism of glycosaminoglycan (GAGs). GAGs incompletely degraded accumulate in many sites, damaging tissues and cells, leading to a variety of clinical manifestation...

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Autores principales: Congedi, Sabrina, Orzalesi, Marcello, Di Pede, Chiara, Benini, Franca
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6213542/
https://www.ncbi.nlm.nih.gov/pubmed/30297617
http://dx.doi.org/10.3390/ijms19103063
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author Congedi, Sabrina
Orzalesi, Marcello
Di Pede, Chiara
Benini, Franca
author_facet Congedi, Sabrina
Orzalesi, Marcello
Di Pede, Chiara
Benini, Franca
author_sort Congedi, Sabrina
collection PubMed
description Mucopolysaccharidosis (MPS) are a group of lysosomal storage disorders that are caused by the deficiency of enzymes involving in the catabolism of glycosaminoglycan (GAGs). GAGs incompletely degraded accumulate in many sites, damaging tissues and cells, leading to a variety of clinical manifestations. Many of these manifestations are painful, but few data are available in the literature concerning the prevalence, etiology, and pathogenesis of pain in children with MPS. This review, through the analysis of the data available the in literature, underscores the relevant prevalence of pain in MPSs’ children, provides the instruments to discern the etiopathogenesis of the disease and of pain, illustrates the available molecules for the management of pain and the possible advantages of non-pharmacological pain therapy in MPSs’ patients.
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spelling pubmed-62135422018-11-14 Pain in Mucopolysaccharidoses: Analysis of the Problem and Possible Treatments Congedi, Sabrina Orzalesi, Marcello Di Pede, Chiara Benini, Franca Int J Mol Sci Review Mucopolysaccharidosis (MPS) are a group of lysosomal storage disorders that are caused by the deficiency of enzymes involving in the catabolism of glycosaminoglycan (GAGs). GAGs incompletely degraded accumulate in many sites, damaging tissues and cells, leading to a variety of clinical manifestations. Many of these manifestations are painful, but few data are available in the literature concerning the prevalence, etiology, and pathogenesis of pain in children with MPS. This review, through the analysis of the data available the in literature, underscores the relevant prevalence of pain in MPSs’ children, provides the instruments to discern the etiopathogenesis of the disease and of pain, illustrates the available molecules for the management of pain and the possible advantages of non-pharmacological pain therapy in MPSs’ patients. MDPI 2018-10-08 /pmc/articles/PMC6213542/ /pubmed/30297617 http://dx.doi.org/10.3390/ijms19103063 Text en © 2018 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Congedi, Sabrina
Orzalesi, Marcello
Di Pede, Chiara
Benini, Franca
Pain in Mucopolysaccharidoses: Analysis of the Problem and Possible Treatments
title Pain in Mucopolysaccharidoses: Analysis of the Problem and Possible Treatments
title_full Pain in Mucopolysaccharidoses: Analysis of the Problem and Possible Treatments
title_fullStr Pain in Mucopolysaccharidoses: Analysis of the Problem and Possible Treatments
title_full_unstemmed Pain in Mucopolysaccharidoses: Analysis of the Problem and Possible Treatments
title_short Pain in Mucopolysaccharidoses: Analysis of the Problem and Possible Treatments
title_sort pain in mucopolysaccharidoses: analysis of the problem and possible treatments
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6213542/
https://www.ncbi.nlm.nih.gov/pubmed/30297617
http://dx.doi.org/10.3390/ijms19103063
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