Williams-Campbell syndrome

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords: Cartilage deficiency

Overview

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]

Historical Perspective

It was described in 1960 by Howard Williams and Peter Campbell.[3][4]

Symptoms

Diagnosis

Radiologically, the lungs are overinflated and on bronchoscopy, bronchomalacia is demonstrated.[2]

References

  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. 2.0 2.1 2.2 2.3 2.4 british medical journal (BMJ)
  3. 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.
  4. 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.

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