Williams–Campbell syndrome

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Williams–Campbell syndrome is a disease of the airways where cartilage in the bronchi is defective. This leads to collapse of the airways and bronchiectasis.[1] Williams–Campbell syndrome is deficiency of the bronchial cartilage distally.[2]

Symptoms[edit]

1. Persistent cough[3]
2. Wheeze[2]
3. Impaired lung function[2]

Diagnosis[edit]

Radiologically, the lungs are overinflated and on bronchoscopy bronchomalacia is demonstrated.[4]

History[edit]

It was described in 1960 by Howard Williams and Peter Campbell.[5][6]

References[edit]

  1. ^ Palmer, S. M.; Layish, D. T.; Kussin, P. S.; Tapson, V. F.; Oury, T.; Davis, R. D. (1998). "Lung Transplantation for Williams-Campbell Syndrome". Chest 113 (2): 534–7. doi:10.1378/chest.113.2.534. PMID 9498979. 
  2. ^ a b c british medical journal (BMJ)
  3. ^ British medical journal (BMJ)
  4. ^ british medical journal
  5. ^ Williams, H; Campbell, P (April 1960). "Generalized Bronchiectasis associated with Deficiency of Cartilage in the Bronchial Tree". Arch. Dis. Child. 35 (180): 182–91. doi:10.1136/adc.35.180.182. PMC 2012546. PMID 13844857. 
  6. ^ Jones, VF; Eid, NS; Franco, SM; Badgett, JT; Buchino, JJ (1993). "Familial congenital bronchiectasis: Williams-Campbell syndrome". Pediatric pulmonology 16 (4): 263–7. doi:10.1002/ppul.1950160410. PMID 8265276.