Williams–Campbell syndrome also known as bronchomalacia is a disease of the airways where cartilage in the bronchi is defective. It is a form of congenital cystic bronchiectasis. This leads to collapse of the airways and bronchiectasis. It acts as one of the differential to Allergic bronchopulmonary aspergillosis. Williams–Campbell syndrome is deficiency of the bronchial cartilage distally.
It is thought to result from a deficiency of cartilage formation in the 4th to 6th order segmental bronchi.
- 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.
- british medical journal (BMJ)
- 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 . PMID 13844857.
- 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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