Allergy Asthma Respir Dis.  2015 Jan;3(1):86-89. 10.4168/aard.2015.3.1.86.

Pulmonary alveolar proteinosis in a 15-year-old girl

Affiliations
  • 1Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea. kmaped@skku.edu
  • 2Department of Thoracic Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
  • 3Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

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.

Keyword

Pulmonary alveolar proteinosis; Child

MeSH Terms

Adolescent*
Child
Female
Granulocytes
Humans
Lipoproteins
Lung Diseases
Macrophage Colony-Stimulating Factor
Macrophages
Prevalence
Pulmonary Alveolar Proteinosis*
Rare Diseases
Surface-Active Agents
Lipoproteins
Macrophage Colony-Stimulating Factor
Surface-Active Agents

Figure

  • Fig. 1 The initial chest radiograph showing ground grass opacities and fine reticular infiltration both upper and middle lobes.

  • Fig. 2 The initial high resolution computed tomography revealing patchy and extensive ground-grass opacity lesions with crazy-paving appearance especially in the upper (A) and middle (B) lung zone.

  • Fig. 3 The histologic findings. (A) Acellular granular eosinophilic proteinaceous materials (arrows) are filled in alveolar space (H&E, ×10). (B) Periodic acid-Schiff staining shows positive (×200).

  • Fig. 4 The follow-up chest radiograph (A) and high resolution computed tomography performed 3 years later from the diagnosis show improvement of patchy and ground glass opacity lesions in both upper (B) and middle (C) lung zone.


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