Primary Ciliary Dyskinesia in Amish Communities

Primary ciliary dyskinesia (PCD) is an autosomal recessive multigenic disease that results in impaired mucociliary clearance. We have diagnosed 9 subjects with primary ciliary dyskinesia from geographically dispersed Amish communities, based on clinical characteristics and ciliary ultrastructural defects. Despite consanguinity, affected individuals had evidence of genetic heterogeneity

Official american thoracic society technical standards: flexible airway endoscopy in children

Background: Flexible airway endoscopy (FAE) is an accepted and frequently performed procedure in the evaluation of children with known or suspected airway and lung parenchymal disorders. However, published technical standards on how to perform FAE in children are lacking. Methods: The American Thoracic Society (ATS) approved the formation of a multidisciplinary committee to delineate technical standards for performing FAE in children. The committee completed a pragmatic synthesis of the evidence and used the evidence synthesis to answer clinically relevant questions. Results: There is a paucity of randomized controlled trials in pediatric FAE. The committee developed recommendations based predominantly on the collective clinical experience of our committee members highlighting the importance of FAE-specific airway management techniques and anesthesia, establishing suggested competencies for the bronchoscopist in training, and defining areas deserving further investigation. Conclusions: These ATS-sponsored technical standards describe the equipment, personnel, competencies, and special procedures associated with FAE in children

Competency-Based Assessment Tool for Pediatric Esophagoscopy: International Modified Delphi Consensus.

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/167424/1/lary29126.pdfhttp://deepblue.lib.umich.edu/bitstream/2027.42/167424/2/lary29126_am.pd

Competency-Based Assessment Tool for Pediatric Tracheotomy: International Modified Delphi Consensus.

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/163458/2/lary28461.pdfhttp://deepblue.lib.umich.edu/bitstream/2027.42/163458/1/lary28461_am.pd