A pediatric case of relapsed pulmonary alveolar proteinosis despite successful whole lung lavage.
10.3345/kjp.2017.60.7.232
- Author:
Seung Young JIN
1
;
Hye Ri YUN
;
Yun Jung CHOI
;
Jun Dong PARK
;
Jin Tae KIM
;
Chang Hyun KANG
;
Young Sik PARK
;
Young Hun CHOI
;
Woo Sun KIM
;
Dong In SUH
Author Information
1. Department of Pediatrics, Seoul National University Hospital, Seoul, Korea. dongins0@snu.ac.kr
- Publication Type:Case Report
- Keywords:
Child;
Pulmonary alveolar proteinosis;
Recurrence;
Therapeutic irrigation;
Video-assisted surgery
- MeSH:
Biopsy;
Bronchoalveolar Lavage*;
Child;
Cough;
Dyspnea;
Humans;
Korea;
Lung*;
Male;
Pulmonary Alveolar Proteinosis*;
Rare Diseases;
Recurrence;
Therapeutic Irrigation;
Video-Assisted Surgery
- From:Korean Journal of Pediatrics
2017;60(7):232-236
- CountryRepublic of Korea
- Language:English
-
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.
