Respiratory Failure of Williams-Campbell Syndrome is Effectively Treated by Noninvasive Positive Pressure Ventilation

Division of Respiratory Medicine, Department of Internal Medicine, Shiga University of Medical Science, Japan.
Internal Medicine (Impact Factor: 0.9). 01/2011; 50(16):1729-32. DOI: 10.2169/internalmedicine.50.4971
Source: PubMed


Williams-Campbell syndrome is a rare disease, characterized by a congenital deficiency of cartilage in the fourth to sixth order bronchi, leading to chronic respiratory failure with recurrent pulmonary infections. An effective and practical treatment has not yet been established. A 31-year-old man who was diagnosed as Williams-Campbell syndrome by inspiratory and expiratory computed tomography findings developed recurrent pulmonary infections and showed progressive deterioration of dyspnea. Domiciliary NPPV was administered, followed by a dramatic improvement of respiratory failure and a decrease in the episodes of pulmonary infections. NPPV may have an advantage in adults with Williams-Campbell syndrome who have severe respiratory failure and recurrent pulmonary infections.

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    • "Hypoxia requires oxygen therapy.14 Noninvasive positive airway pressure can be used when respiratory acidosis exists.16 Other acquired and congenital conditions associated with bronchiectasis, including ciliary dyskinesia, cystic fibrosis, allergic bronchopulmonary aspergillosis, and immunoglobin deficiencies, must be excluded. "
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    ABSTRACT: Williams-Campbell syndrome, also known as bronchomalacia, is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi, leading to distal airway collapse and bronchiectasis. There have been few reports about patients affected by saccular bronchiectasis, paracicatricial emphysema, and diminished cartilage. These are all characteristic of Williams-Campbell syndrome. This report presents a 57-year-old woman with progressive dyspnea, cough, sputum production, and fever. The clinical and laboratory examination revealed that the patient had a respiratory infection due to bronchiectasis caused by Williams-Campbell syndrome, which was undiagnosed in the patient until then. Although a rare syndrome, when patients' signs and symptoms include recurrent respiratory infections, bronchiectasis, productive cough, and dyspnea, Williams-Campbell syndrome should be included in the differential diagnosis.
    International Journal of General Medicine 01/2012; 5:41-4. DOI:10.2147/IJGM.S28447
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    ABSTRACT: Williams-Campbell syndrome is a rare disorder characterized by deficiency of subsegmental bronchial cartilage and development of airway collapse and bronchiectasis that may subsequently progress to respiratory failure and death. There are only 2 published reports suggesting a familial association, and only one report of lung transplantation being used as a therapeutic modality. Due to postoperative airway complications, transplantation has not been recommended for this disease. We report the first lung transplant with prolonged survival, approaching 10 years, in a patient with Williams-Campbell syndrome, and provide further evidence to support a familial association.
    Respiratory care 02/2012; 57(9):1505-8. DOI:10.4187/respcare.01484 · 1.84 Impact Factor
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    ABSTRACT: Williams-Campbell syndrome is a rare congenital syndrome characterized by the absence of cartilage in subsegmental bronchi leading to formation of bronchiectasis distal to the affected bronchi. The differential diagnosis of bronchiectasis is broad and the rarity of the disease poses a diagnostic and management challenge for clinicians. This present review aims to help the understanding of the clinical manifestations, pathophysiological features, diagnostic modalities, management and differential diagnosis of Williams-Campbell syndrome. A MedLine/PubMed search was performed identifying all relevant articles. No restrictions were used for publication dates. The author used the keywords "Williams-Campbell syndrome," "non-cystic fibrosis bronchiectasis" and "congenital bronchiectasis" finding 503, 195 and 489 articles, respectively.
    North American Journal of Medical Sciences 09/2014; 6(9):429-432. DOI:10.4103/1947-2714.141620