Pulmonary alveolar proteinosis in a 15-year-old girl.
- Author:
Yechan KYUNG
1
;
Jihyun KIM
;
Hong Kwan KIM
;
Joungho HAN
;
Kangmo AHN
Author Information
- Publication Type:Case Report
- Keywords: Pulmonary alveolar proteinosis; Child
- MeSH: Adolescent*; Child; Female; Granulocytes; Humans; Lipoproteins; Lung Diseases; Macrophage Colony-Stimulating Factor; Macrophages; Prevalence; Pulmonary Alveolar Proteinosis*; Rare Diseases; Surface-Active Agents
- From:Allergy, Asthma & Respiratory Disease 2015;3(1):86-89
- CountryRepublic of Korea
- Language:Korean
- Abstract: Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease characterized by the accumulation of lipoproteins derived from surfactants in the distal air space. The lack of granulocyte macrophage colony-stimulating factor is believed to contribute to macrophage dysfunction and the impaired processing of surfactants. Because the prevalence of PAP in the general population is less than 1 in 200,000, and the typical age at presentation is 35 to 50 years, PAP is a very rare disease in children. To the best of our knowledge, there has been no Korean report on PAP in children. We describe here a patient who was diagnosed with PAP at the aged 15 years.
