Successful pregnancy in a severely hypoxemic patient with pulmonary alveolar proteinosis

Belchior, I.; Cerdeira, A.; Santos, M.; Braga, J.; Aragâo, I.; Martins, A.

http://hdl.handle.net/10400.16/869

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Abstract(s)

Abstract Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by abnormal accumulation of a lipoproteinaceous material in the alveoli, which may lead to respiratory failure and has an associated high risk for infections. The mainstay treatment for PAP is whole lung lavage. A pregnant woman, previously diagnosed with primary PAP, the most common form of PAP, was admitted with dyspnea and worsening respiratory function. In one month period, a wholelung bronchopulmonary lavage was performed twice, with clinical and functional improvement. Pregnancy was carried to term and a healthy baby was delivered. The mechanisms of respiratory impairment are discussed as well as treatment options and response. Resumo A proteinose alveolar pulmonar (PAP) é uma doença rara caracterizada pela acumulação anormal de material lipoproteináceo nos alvéolos, que pode levar a insufi ciência respiratória, estando associada a um risco elevado de infecções. O tratamento gold-standard da PAP é a lavagem pulmonar total.

Keywords

Pregnancy Pulmonary alveolar proteinosis Pulmonary lavage Respiratory failure Gravidez Proteinose alveolar Pulmonar Lavagem pulmonar Insuficiência respiratória