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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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2018
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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. |
format | Online Article Text |
id | pubmed-6213542 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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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