Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2018 Oct 8;19(10):3063.
doi: 10.3390/ijms19103063.

Pain in Mucopolysaccharidoses: Analysis of the Problem and Possible Treatments

Sabrina Congedi  1 , Marcello Orzalesi  2 , Chiara Di Pede  3 , Franca Benini  4   5
Affiliations
Review

Pain in Mucopolysaccharidoses: Analysis of the Problem and Possible Treatments

Sabrina Congedi et al. Int J Mol Sci. .

Abstract

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.

Keywords: children; muchopolysaccaridoses; pain; treatments.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

References

    1. Neufeld E.F., Muenzer J. The Online Metabolic and Molecular Bases of Inherited Disease. McGraw-Hill Medical; New York, NY, USA: 2014. The Mucopolysaccharidoses.
    1. Cassis L., Cortès-Saladelafont E., Molero-Luis M., Yubero D., González M.J., Ormazábal A., Fons C., Jou C., Sierra C., Castejon Ponce E., et al. Review and evaluation of the methodological quality of the existing guidelines and recommendations for inherited neurometabolic disorders. Orphanet J. Rare Dis. 2015;10:164. doi: 10.1186/s13023-015-0376-9. - DOI - PMC - PubMed
    1. Baehner F., Schmiedeskamp C., Krummenauer F., Miebach E., Bajbouj M., Whybra C., Kohlschütter A., Kampmann C., Beck M. Cumulative incidence rates of the mucopolysaccharidoses in Germany. J. Inherit. Metab. Dis. 2005;28:1011–1017. doi: 10.1007/s10545-005-0112-z. - DOI - PubMed
    1. Nelson J., Crowhurst J., Carey B., Greed L. Incidence of the mucopolysaccharidoses in Western Australia. Am. J. Med. Genet. 2003;123A:310–313. doi: 10.1002/ajmg.a.20314. - DOI - PubMed
    1. Ben Turkia H., Tebib N., Azzouz H., Abdelmoula M.S., Ben Chehida A., Chemli J., Monastiri K., Chaabouni M., Sanhagi H., Zouari B., et al. Incidence of mucopolysaccharidoses in Tunisia. Tunis. Med. 2009;87:782–785. - PubMed