[Alveolar proteinosis. Report of 7 cases (author's transl)].
We followed 7 patients with biopsy proven alveolar proteinosis for 4 to 96 months. A typical clinical picture including: dyspnea, cyanosis, weight loss, clubbing, and rales, was present in 6 subjects. The 7th subject presented repeated bronchial infections. Chest Roentgenogram revealed an alveolar type infiltrate in each case. Sputum examination showed alveolar moulds of PAS positive material. Spontaneous improvement occurred in one subject who had only a moderate airway obstruction. The others, with severe restrictive defects, were rapidly improved by massive pulmonary lavage. Continued improvement was noted in the months following lavage. Repeated lavages was required in three subjects. Although spontaneous recovery of this rare disorder may occur, severe cases require pulmonary lavage. The diagnosis of alveolar proteinosis can be confirmed by analysis of sputum or lavage material.
D000328:Adult; D002648:Child; D005260:Female; D006801:Humans; D008168:Lung / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D007507:Therapeutic Irrigation
La protéinose alvéolaire. A propos de 7 cas.