Cystic fibrosis and Chiari type I malformation: autopsy study of two infants with a rare association.

@article{Rakheja2003CysticFA,
  title={Cystic fibrosis and Chiari type I malformation: autopsy study of two infants with a rare association.},
  author={Dinesh Rakheja and Yin Xu and Dennis K. Burns and Daniel L Veltkamp and Linda R. Margraf},
  journal={Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society},
  year={2003},
  volume={6 1},
  pages={88-93}
}
Cystic fibrosis (CF), an epithelial cell transport disorder caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene, is not generally associated with malformations of the central nervous system (CNS). This report describes two African-American children who died at less than 2 years of age with known Chiari I malformations and were found, unexpectedly at autopsy, to have the classic pancreatic and respiratory changes of CF. Both patients had suffered from… CONTINUE READING