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KMID : 1130320170600070232
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Korean Journal of Pediatrics
2017 Volume.60 No. 7 p.232 ~ p.236
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A pediatric case of relapsed pulmonary alveolar proteinosis despite successful whole lung lavage
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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
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Abstract
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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.
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KEYWORD
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Child, Pulmonary alveolar proteinosis, Recurrence, Therapeutic irrigation, Video-assisted surgery
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