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Korean J Pediatr 2017 July;60(7) :232-236.
Published online 2017 July 15.       
A pediatric case of relapsed pulmonary alveolar proteinosis despite successful whole lung lavage
Seung Young Jin1, Hye Ri Yun1, Yun Jung Choi1, Jun Dong Park1, Jin Tae Kim2, Chang Hyun Kang3, Young Sik Park4, Young Hun Choi5, Woo Sun Kim5, Dong In Suh1
1Department of Pediatrics, Seoul National University Hospital, Seoul, Korea
2Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul, Korea
3Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul, Korea
4Department of Internal Medicine and Lung Institute, Seoul National University Hospital, Seoul, Korea
5Department of Radiology, Seoul National University Hospital, Seoul, Korea
Corresponding Author: Dong In Suh ,Tel: +82-2-2072-3625, Fax: +82-2-743-3455, Email: dongins0@snu.ac.kr
Copyright © 2017 by The Korean Pediatric Society
ABSTRACT
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.
Keywords: Child | Pulmonary alveolar proteinosis | Recurrence | Therapeutic irrigation | Video-assisted surgery
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