This Case Report is that of a young woman followed for 21 years, whose life was saved repeatedly by bronchoscopy and lavage to remove mucous plugs and casts from the tracheobronchial tree. In 1982, after again having a respiratory crisis and not having a bronchoscopy and lavage, she died. The pathology revealed a thickened hyalinized basement membrane and numerous mucous plugs filling the larger bronchi and bronchioles.
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Millman et al. (1985) studied this question.
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