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

Affiliations
  • 1Department of Pediatrics, Seoul National University Hospital, Seoul, Korea. dongins0@snu.ac.kr
  • 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.

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
Full Text Links
  • KJP
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr