Abstract
BackgroundPulmonary alveolar proteinosis is a rare disease characterized by accumulation of lipoproteinaceous material within alveoli. There are three clinically distinct forms: congenital, acquired and secondary. Whole lung lavage is currently the gold standard therapy for severe cases of pulmonary alveolar proteinosis. In Sri Lanka this is the first reported successful whole lung lavage for a patient with pulmonary alveolar proteinosis.Case presentationWe describe the case of a 15-year-old Sri Lankan girl who presented with symptoms of progressive shortness of breath and dry cough for 6 months’ duration. She had a history of exposure to silica in her household environment. High-resolution computed tomography revealed crazy paving appearance in both lungs suggestive of pulmonary alveolar proteinosis. An open lung biopsy revealed intra-alveolar granular amphophilic material which was strongly periodic acid–Schiff positive and diastase resistant, which is consistent with pulmonary alveolar proteinosis. She was followed up for 2 years with periodical segmental bronchoalveolar lavages which showed minimal improvement in her symptoms as well as in exercise desaturation. Due to severe dyspnea and hypoxemia on exertion, she underwent whole lung lavage. It resulted in a marked improvement in her symptoms, exercise desaturation, and chest X-ray results.ConclusionWhole lung lavage was successfully performed for the first time in Sri Lanka for a patient with pulmonary alveolar proteinosis.
Highlights
BackgroundPulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of a lipoproteinaceous, eosinophilic, periodic acid–Schiff (PAS) positive material within the alveoli
Pulmonary alveolar proteinosis is a rare disease characterized by accumulation of lipoproteinaceous material within alveoli
Whole lung lavage was successfully performed for the first time in Sri Lanka for a patient with pulmonary alveolar proteinosis
Summary
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of a lipoproteinaceous, eosinophilic, periodic acid–Schiff (PAS) positive material within the alveoli. Development of lung involvement and diagnosis of PAP In 2014 at 15 years of age, she presented to our clinic with a 6-month history of progressive dyspnea which limited her exercise capacity. She noticed that she started becoming breathless while she walked from the school gate to the classroom carrying her school bag. She underwent seven episodes of therapeutic segmental BAL over a period of 1.5 years from March 2014 to August 2015 At each episode, she was carefully assessed for fitness for the procedure with the indication of exercise desaturation and progressive dyspnea. Our patient was observed in the ICU for approximately 12
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