Recalcitrant chronic rhinosinusitis in the setting of fucosidosis, a rare lysosomal storage disorder Article Conference Paper uri icon

Overview

MeSH Major

  • Leukemia, Lymphocytic, Chronic, B-Cell
  • Oropharyngeal Neoplasms
  • Paranasal Sinus Neoplasms
  • Paranasal Sinuses
  • Sex Factors

abstract

  • © 2017 Elsevier B.V. Fucosidosis is an autosomal recessive lysosomal storage disorder caused by the deficiency of alpha-L-fucosidase. We present the case of an affected female in the second decade of life with chronic rhinosinusitis (CRS) including recalcitrant polypoid inflammation, which has not been previously reported in the literature. With the advancement of life-prolonging measures, children with lysosomal storage disorders may suffer increasingly from CRS due to the lymphohistiocytic and macrophage infiltrate of the paranasal sinus mucosa that resembles severe polypoid inflammation.

publication date

  • December 2017

Research

keywords

  • Conference Paper

Identity

Digital Object Identifier (DOI)

  • 10.1016/j.ijporl.2017.09.019

PubMed ID

  • 29224764

Additional Document Info

start page

  • 5

end page

  • 9

volume

  • 103