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Pulmonary alveolar proteinosis: four cases
João Carlos Thompson1, Marina Kishima, Mariana Ulbricht Gomes
1Royal Brompton Hospital, London, England. zthomson@sercomtel.com.br
Whole-lung lavage is an effective treatment for pulmonary alveolar proteinosis (PAP). While some patients show resistance, this procedure can lead to complete disease remission in others.
Area of Science:
- Pulmonology
- Thoracic Surgery
- Critical Care Medicine
Background:
- Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of surfactant-like material in the alveoli.
- This study examines the clinical course and treatment outcomes of four PAP patients.
Observation:
- Four patients (3 female, 1 male, aged 22-34) with progressive dyspnea and dry cough were diagnosed with PAP via open-lung biopsy.
- Three patients underwent whole-lung lavage (WLL) under general anesthesia with a double-lumen endotracheal tube.
Findings:
- One patient experienced spontaneous regression of PAP without intervention.
- Of the three patients who underwent WLL, one achieved complete remission after a single unilateral lavage, while another showed no significant improvement after three lavages.
- The fourth patient required four lavages, experiencing only transient improvements.
Implications:
- Whole-lung lavage is a valuable therapeutic option for pulmonary alveolar proteinosis.
- Treatment response to WLL can vary, with some patients demonstrating resistance despite multiple procedures.
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