Williams-Campbell syndrome: Difference between revisions

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'''Williams–Campbell syndrome''' is a [[disease]] of the [[airway]]s where [[cartilage]] in the [[bronchus|bronchi]] is defective. This leads to collapse of the airways and [[bronchiectasis]].<ref name="Palmer">{{cite journal |pages=534–7 |doi=10.1378/chest.113.2.534 |title=Lung Transplantation for Williams-Campbell Syndrome |year=1998 |last1=Palmer |first1=S. M. |last2=Layish |first2=D. T. |last3=Kussin |first3=P. S. |last4=Tapson |first4=V. F. |last5=Oury |first5=T. |last6=Davis |first6=R. D. |journal=Chest |volume=113 |issue=2 |pmid=9498979}}</ref>
'''Williams–Campbell syndrome''' is a [[disease]] of the [[airway]]s where [[cartilage]] in the [[bronchus|bronchi]] is defective. This leads to collapse of the airways and [[bronchiectasis]].<ref name="Palmer">{{cite journal |pages=534–7 |doi=10.1378/chest.113.2.534 |title=Lung Transplantation for Williams-Campbell Syndrome |year=1998 |last1=Palmer |first1=S. M. |last2=Layish |first2=D. T. |last3=Kussin |first3=P. S. |last4=Tapson |first4=V. F. |last5=Oury |first5=T. |last6=Davis |first6=R. D. |journal=Chest |volume=113 |issue=2 |pmid=9498979}}</ref>
Williams–Campbell syndrome is deficiency of the [[bronchial]] [[cartilage]] distally.<ref name="Britishmedical">[[british medical journal]] (BMJ)</ref>
Williams–Campbell syndrome is deficiency of the [[bronchial]] [[cartilage]] distally.<ref name="Britishmedical">[[british medical journal]] (BMJ)</ref>
==Historical Perspective==
It was described in 1960 by Howard Williams and Peter Campbell.<ref name="pmid13844857">{{cite journal |author1=Williams, H | author2 = Campbell, P |title=Generalized Bronchiectasis associated with Deficiency of Cartilage in the Bronchial Tree |journal=Arch. Dis. Child. |volume=35 |issue= 180|pages=182–91 |date=April 1960 |pmid=13844857 |pmc=2012546 |doi= 10.1136/adc.35.180.182|url=}}</ref><ref>{{cite journal |pmid=8265276 |year=1993 |last1=Jones |first1=VF |last2=Eid |first2=NS |last3=Franco |first3=SM |last4=Badgett |first4=JT |last5=Buchino |first5=JJ |title=Familial congenital bronchiectasis: Williams-Campbell syndrome |volume=16 |issue=4 |pages=263–7 |journal=Pediatric pulmonology |doi=10.1002/ppul.1950160410}}</ref>


==Symptoms==
==Symptoms==
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==Diagnosis==
==Diagnosis==
Radiologically, the lungs are [[Hyperinflation|overinflated]] and on [[bronchoscopy]] [[bronchomalacia]] is demonstrated.<ref name="Britishmedical" />
Radiologically, the lungs are [[Hyperinflation|overinflated]] and on [[bronchoscopy]], [[bronchomalacia]] is demonstrated.<ref name="Britishmedical" />
 
==History==
It was described in 1960 by Howard Williams and Peter Campbell.<ref name="pmid13844857">{{cite journal |author1=Williams, H | author2 = Campbell, P |title=Generalized Bronchiectasis associated with Deficiency of Cartilage in the Bronchial Tree |journal=Arch. Dis. Child. |volume=35 |issue= 180|pages=182–91 |date=April 1960 |pmid=13844857 |pmc=2012546 |doi= 10.1136/adc.35.180.182|url=}}</ref><ref>{{cite journal |pmid=8265276 |year=1993 |last1=Jones |first1=VF |last2=Eid |first2=NS |last3=Franco |first3=SM |last4=Badgett |first4=JT |last5=Buchino |first5=JJ |title=Familial congenital bronchiectasis: Williams-Campbell syndrome |volume=16 |issue=4 |pages=263–7 |journal=Pediatric pulmonology |doi=10.1002/ppul.1950160410}}</ref>


==References==
==References==

Latest revision as of 16:44, 20 January 2016

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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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