Chronic airflow obstruction in Fabry's disease

David M. Rosenberg, Victor J. Ferrans, Jack D. Fulmer, Bruce R. Line, John A. Barranger, Roscoe O. Brady, Ronald Crystal

Research output: Contribution to journalArticle

50 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)898-905
Number of pages8
JournalThe American Journal of Medicine
Volume68
Issue number6
DOIs
Publication statusPublished - 1 Jan 1980
Externally publishedYes

    Fingerprint

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Rosenberg, D. M., Ferrans, V. J., Fulmer, J. D., Line, B. R., Barranger, J. A., Brady, R. O., & Crystal, R. (1980). Chronic airflow obstruction in Fabry's disease. The American Journal of Medicine, 68(6), 898-905. https://doi.org/10.1016/0002-9343(80)90224-7