Chronic airflow obstruction in Fabry's disease.

  title={Chronic airflow obstruction in Fabry's disease.},
  author={David M. Rosenberg and V{\'i}ctor J. Ferrans and Jack D. Fulmer and Bruce R. Line and John A. Barranger and Roscoe O. Brady and Ronald G. Crystal},
  journal={The American journal of medicine},
  volume={68 6},
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… CONTINUE READING


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