Respirology : official journal of the Asian Pacific Society of Respirology
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The objective of this study was to present clinical and radiological data of eight women with histologically proven lymphangioleiomyomatosis (LAM) diagnosed between 1984 and 1994, and to suggest a diagnostic strategy when LAM is suspected. A review of case reports, including results of biopsies, lung function and radiological procedures was undertaken. The mean age of the women at start of symptoms was 36 years, and the mean age at time of diagnosis 42 years. ⋯ Three specimens had to be revised before the histological diagnosis was confirmed. It was concluded that the important clues to a diagnosis of LAM are recurrent episodes of pneumothoraces in fertile women, progressive air-flow limitation, markedly reduced gas transfer and characteristic findings on thoracic CT scans. A specific request to the pathologist to stain lung tissue specimens for smooth muscle cells is mandatory.
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Clinical Trial
Treatment of severe exacerbation of chronic obstructive pulmonary disease with mask-applied continuous positive airway pressure.
The efficacy of mask-applied continuous positive airway pressure (CPAP) in the treatment of patients with acute severe exacerbations of chronic obstructive pulmonary disease (COPD) was examined. Ten patients with severe exacerbation of COPD who had deteriorated during conventional therapy were treated with face-mask delivered CPAP (+5 cmH2O; Downs Vital signs Inc., New Jersey, USA) instead of tracheal intubation and mechanical ventilation. The patients that were selected required mental alertness, intact upper airway reflexes, the clinical signs of dynamic hyperinflation and a positive end-expiratory pressure auto-(PEEP) manifested as expiratory wheeze and grunting. ⋯ One patient deteriorated on mask-CPAP and required intubation and mechanical ventilation. Three patients died (none of these patients died during the acute period of exacerbation). It was concluded that Mask-CPAP may be an alternative to mechanical ventilation in the treatment of selected patients with severe hypercapnic exacerbations of COPD.