Chronic airflow obstruction in Fabry's disease. Academic Article uri icon

Overview

MeSH

  • Adult
  • Epithelium
  • Female
  • Galactosylgalactosylglucosylceramidase
  • Humans
  • Inclusion Bodies
  • Male
  • Middle Aged
  • Pulmonary Alveoli
  • Respiratory Function Tests
  • Smoking
  • Ventilation-Perfusion Ratio

MeSH Major

  • Airway Obstruction
  • Fabry Disease

abstract

  • Seven patients with Fabry's disease, an x-linked sphingolipid storage disorder, were evaluated for the presence and extent of airway obstruction. All were found to have significant obstruction to airflow. In addition, evaluation of their airway epithelial cells obtained by bronchoscopy demonstrated that these cells contained inclusion bodies consistent with deposits of ceramide trihexoside, suggesting that part of their functional obstruction to airflow may be secondary to intrinsic airway disease. Although all of the study population had evidence of airflow obstruction, the impairment was much worse in those who smoked, implying that even mild cigarette smoking is particularly hazardous to patients with Fabry's disease.

publication date

  • June 1980

has subject area

  • Adult
  • Airway Obstruction
  • Epithelium
  • Fabry Disease
  • Female
  • Galactosylgalactosylglucosylceramidase
  • Humans
  • Inclusion Bodies
  • Male
  • Middle Aged
  • Pulmonary Alveoli
  • Respiratory Function Tests
  • Smoking
  • Ventilation-Perfusion Ratio

Research

keywords

  • Journal Article

Identity

Language

  • eng

PubMed ID

  • 6247911

Additional Document Info

start page

  • 898

end page

  • 905

volume

  • 68

number

  • 6