Familial occurrence is rare. The pathogenesis of the bronchiectasia is unclear. The lymphedema is thought to result from hypoplasia of the lymphatics. The pleural effusions are exudative and there is dilatation of both visceral and parietal pleural lymphatics with perilymphatic inflammation. The radiograph shows evidence of hyperinflation and demonatrate non-specific opacities.
Bronchial disease occurs frequently and is an integral feature of the yellow nail syndrome.
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