Williams–Campbell syndrome

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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.[1] It acts as one of the differential to Allergic bronchopulmonary aspergillosis. Williams–Campbell syndrome is deficiency of the bronchial cartilage distally.[2]

Symptoms[edit]

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

Pathology[edit]

It is thought to result from a deficiency of cartilage formation in the 4th to 6th order segmental bronchi.

Diagnosis[edit]

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

History[edit]

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

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. PMID 9498979. doi:10.1378/chest.113.2.534. 
  2. ^ a b c d e 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. PMC 2012546Freely accessible. PMID 13844857. doi:10.1136/adc.35.180.182. 
  4. ^ Jones, VF; Eid, NS; Franco, SM; Badgett, JT; Buchino, JJ (1993). "Familial congenital bronchiectasis: Williams-Campbell syndrome". Pediatric pulmonology. 16 (4): 263–7. PMID 8265276. doi:10.1002/ppul.1950160410.