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The Complexity of Pain Management in Children Affected by Mucopolysaccharidoses

Mucopolysaccharidoses (MPSs) are a group of rare, genetic lysosomal storage disorders. They are caused by deficiencies of the lysosomal enzymes involved in the degradation of glycosaminoglycans (GAGs). Pain is a common feature in mucopolysaccharidoses. However, the pathophysiology of pain in this gr...

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Detalles Bibliográficos
Autores principales: Congedi, Sabrina, Di Pede, Chiara, Scarpa, Maurizio, Rampazzo, Angelica, Benini, Franca
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5394408/
https://www.ncbi.nlm.nih.gov/pubmed/28473938
http://dx.doi.org/10.1155/2017/7257230
Descripción
Sumario:Mucopolysaccharidoses (MPSs) are a group of rare, genetic lysosomal storage disorders. They are caused by deficiencies of the lysosomal enzymes involved in the degradation of glycosaminoglycans (GAGs). Pain is a common feature in mucopolysaccharidoses. However, the pathophysiology of pain in this group of diseases is still unclear and genesis of pain is multifactorial. Currently, poor data about pain management in these patients are available. Here, we present our clinical experience in complex pain management in three children with MPS.