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A pediatric case of relapsed pulmonary alveolar proteinosis despite successful whole lung lavage

Pulmonary alveolar proteinosis (PAP) is a rare disease in children characterized by intra-alveolar accumulation of surfactant proteins, which severely reduces gaseous exchange. Whole lung lavage (WLL) is the preferred technique for the treatment of severe PAP. Herein, we present a pediatric case of...

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Detalles Bibliográficos
Autores principales: Jin, Seung Young, Yun, Hye Ri, Choi, Yun Jung, Park, Jun Dong, Kim, Jin Tae, Kang, Chang Hyun, Park, Young Sik, Choi, Young Hun, Kim, Woo Sun, Suh, Dong In
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Pediatric Society 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5573747/
https://www.ncbi.nlm.nih.gov/pubmed/28861115
http://dx.doi.org/10.3345/kjp.2017.60.7.232
Descripción
Sumario:Pulmonary alveolar proteinosis (PAP) is a rare disease in children characterized by intra-alveolar accumulation of surfactant proteins, which severely reduces gaseous exchange. Whole lung lavage (WLL) is the preferred technique for the treatment of severe PAP. Herein, we present a pediatric case of PAP treated with WLL. An 11-year-old boy was admitted with the chief complaint of a dry cough lasting 6 months. He developed symptoms of dyspnea on exertion and had difficulty in climbing stairs. He was ultimately diagnosed with PAP through video-assisted thoracoscopic lung biopsy. As first-line of treatment for PAP, he underwent therapeutic WLL for each of his lungs on separate days. After a brief recovery, his symptoms gradually worsened; therefore, he underwent a second WLL. This is the first pediatric case of PAP relapse despite successful WLL in Korea.