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15세 여아에서 발생한 폐포단백증 1예
- Issue Date
- 2015
- Publisher
- 대한 소아알레르기 호흡기학회
- Keywords
- Pulmonary alveolar proteinosis; Child
- Citation
- Allergy Asthma & Respiratory Diseases, v.3, no.1, pp.86 - 91
- Indexed
- KCI
- Journal Title
- Allergy Asthma & Respiratory Diseases
- Volume
- 3
- Number
- 1
- Start Page
- 86
- End Page
- 91
- ISSN
- 2288-0402
- 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.
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