PMC5458054_01
Anterior posterior view of the chest taken in the emergency department, demonstrating left-sided pneumothorax with lung collapse.
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Chest X-ray was performed, which showed a left-sided pneumothorax with lung collapse (Fig. 1).
CT images of the chest and flexible bronchoscopy findings. A-C: HRCT shows bronchiectasis in the left lower lobe, opacities in both lower lungs, and bilateral panacinar emphysema. D: Flexible bronchoscopic findings 1 week after complete lung expansion, revealing a large amount of yellowish bronchial secretions from bilateral bronchi. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
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High-resolution computed tomography (HRCT) of the chest was performed, which showed fully expanded lungs with left lower lobe bronchiectasis, bronchial wall thickening, patchy bilateral ground-glass opacities, and bilateral panacinar emphysema. (Fig. 2A-C).
Electron microscopy of cilia from bronchial mucosa. A, B: Transmission electron micrograph of cross-section of cilia shows absence of both inner and outer dynein arms, and transposition defect: some cilia demonstrate absence of a central microtubule pair '9 + 0'(black arrows); in other cilia a peripheral microtubule doublet has crossed to take the central position providing an apparent '8 + 2' structure (black arrows) and microtubular disorganization. C: Cilia of bronchial epithelia were sparse and presented with local defects. D-F: Scanning electron microscopy showed loss of surface cilia, and a large number of red blood cells and mucinous structures adhered to the surface of the mucous membranes.
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Flexible bronchoscopy at 16 days after admission revealed a massive amount of yellowish bronchial secretions from the right middle lobe bronchus (Fig. 3D). Electron microscopic examination of biopsy specimens obtained from the bronchial mucosa revealed a combined defect of both IDA and ODA in the cilia and disorganization of doublet distribution (Fig. 3B).