Korean J Pediatr.  2017 Jul;60(7):232-236. 10.3345/kjp.2017.60.7.232.

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

• Jin SY
• Yun HR
• Choi YJ
• Park JD
• Kim JT
• Kang CH
• Park YS
• Choi YH
• Kim WS
• Suh DI

• KMID: 2387773
• DOI: http://doi.org/10.3345/kjp.2017.60.7.232

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.

Keyword

Child; Pulmonary alveolar proteinosis; Recurrence; Therapeutic irrigation; Video-assisted surgery

MeSH Terms

Biopsy
Bronchoalveolar Lavage*
Child
Cough
Dyspnea
Humans
Korea
Lung*
Male
Pulmonary Alveolar Proteinosis*
Rare Diseases
Recurrence
Therapeutic Irrigation
Video-Assisted Surgery