We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Williams-Campbell Syndrome Diagnosed in Adulthood: A Rare Entity of Bronchiectasis.
- Authors
Kılınçarslan, Kübra Gül; Akyıl, Fatma Tokgöz; Boyracı, Neslihan; Sucu, Metin; Arslan, Melih Akay; Abalı, Hülya; Kara, Kaan; Önür, Seda Tural
- Abstract
Williams-Campbell syndrome is a rare condition caused by a deficiency or complete absence of bronchial wall cartilage formation, resulting in distal airway collapse and bronchiectasis. The syndrome, generally encountered first during infancy, progresses with recurrent pneumonia with such obstructive symptoms as cough and wheezing from childhood onwards, while adult cases are rarely reported. A 40-year-old male patient presented with complaints of shortness of breath, cough and fever for one week. He was hospitalized with a diagnosis of bronchiectasis exacerbation due to elevated infection parameters, when radiology revealed extensive cystic bronchiectasis, air trapping and airway collapse at expiration. After excluding other etiologies, the patient was diagnosed with Williams-Campbell syndrome in light of the clinical and radiologic findings. He was discharged following clinical response and started on a pulmonary rehabilitation program, was referred to a lung transplantation clinic. The case is representative of the set of rare adult cases showing that Williams-Campbell syndrome can be diagnosed in adulthood, and signaling that the etiology of bronchiectasis should be further evaluated in advanced ages.
- Subjects
BRONCHIECTASIS; ADULTS; SYNDROMES; DYSPNEA; LUNG transplantation; DIAGNOSIS
- Publication
Respiratory Case Reports, 2022, Vol 11, Issue 1, p50
- ISSN
2147-2475
- Publication type
Article
- DOI
10.5505/respircase.2022.94546