Document Detail

Bronchoscopic evaluation of airway obstruction in campomelic dysplasia.
MedLine Citation:
PMID:  3670910     Owner:  NLM     Status:  MEDLINE    
Campomelic dysplasia is a generalized disorder of cartilaginous growth and development, leading to early death from pulmonary insufficiency. We describe the airway dynamics as observed bronchoscopically in two affected infants. Both infants demonstrated anatomic compromise of the upper airway and diffuse laryngotracheobronchomalacia. Additionally, both had a characteristically small, bell-shaped thoracic cage. The abnormal airway dynamics produced serious inspiratory and expiratory obstruction in these infants and, in combination with the restrictive chest wall defect, led rapidly to the development of respiratory failure. While palliative procedures such as tracheostomy may temporarily improve airway dynamics, future respiratory tract insults may prove fatal.
R Grad; P H Sammut; J R Britton; P Goodrich; H E Hoyme; N N Dambro
Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Pediatric pulmonology     Volume:  3     ISSN:  8755-6863     ISO Abbreviation:  Pediatr. Pulmonol.     Publication Date:    1987 Sep-Oct
Date Detail:
Created Date:  1987-12-17     Completed Date:  1987-12-17     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  8510590     Medline TA:  Pediatr Pulmonol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  364-7     Citation Subset:  IM    
Department of Pediatrics, University of Arizona College of Medicine, Tucson 85724.
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MeSH Terms
Airway Obstruction / pathology*
Bronchi / pathology*
Fiber Optic Technology
Infant, Newborn
Osteochondrodysplasias / pathology*
Grant Support

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