Pulmonary alveolar proteinosis (PAP) is an orphan disease characterized by the accumulation of lipoproteinaceous material in the alveoli due to macrophage dysfunction, leading to impaired gas exchange and hypoxemia of variable severity. Diagnosis is made by the constellation of clinical signs and radiological findings supported by bronchoalveolar lavage (BAL) or transbronchial lung biopsy reports. Whole lung lavage (WLL) is the treatment of choice. Here, we report a case of a middle-aged female diagnosed of having PAP based on BAL findings and treated with WLL.
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Tale et al. (2024) studied this question.
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