Case study: Patient with alveolar proteinosis

Wessels S.P. (1991)


Alveolar proteinosis is a rare disease of unknown etiology in which the alveoli are filled with lipid-proteinaceous material. The diagnosis is usually made on an open lung biopsy. The main symptoms are chest pain, tiredness, persistent pyrexia and a productive cough with purulent, bloodstained sputum. Exertion dyspnoea, however, is regarded as the most important symptom. Chest X-rays demonstrate a picture similar to severe pulmonary oedema. The lung functions and the blood gases are indicators of the severity of the disease and there is usually a reduction in the vital capacity, SaO2 and PaO2. A case study of a patient with this diagnosis treated at Tygerberg Hospital is reported. He was admitted complaining of the above mentioned symptoms, his lung functions and blood gases were extremely poor and the chest X-rays showed bilateral diffuse opacifications. He had to be ventilated with high percentages of oxygen and a high PEEP to maintain an adequate SaO2 and PaO2. The infection was treated with antibiotics and corticosteroids. Because of the presence of a tremendous amount of bronchial secretions, chest physiotherapy was very important. General techniques used were bagging, percussion and shaking in alternative side-lying and were combined with Mistabron:Saline inhalations to help dissolve the proteinaceous material in the alveoli. Over a period of 3 weeks he recovered remarkably, but two months after admission his condition unexpectedly deteriorated and he died three days later.

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