Pulmonary alveolar proteinosis in a 15-year-old girl

Yechan Kyung; Jihyun Kim; Hong Kwan Kim; Joungho Han; Kangmo Ahn

doi:10.4168/aard.2015.3.1.86

Journal List > Allergy Asthma Respir Dis > v.3(1) > 1059156

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Kyung, Kim, Kim, Han, and Ahn: Pulmonary alveolar proteinosis in a 15-year-old girl

Case Report

Allergy, Asthma & Respiratory Disease 2015; 3(1): 86-89.

Published online: 31 January 2015

DOI: https://doi.org/10.4168/aard.2015.3.1.86

Pulmonary alveolar proteinosis in a 15-year-old girl

Yechan Kyung¹, Jihyun Kim¹, Hong Kwan Kim², Joungho Han³, Kangmo Ahn¹

¹Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

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.

Correspondence to: Kangmo Ahn. Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul 135-710, Korea. Tel: +82-2-3410-3530, Fax: +82-2-3410-0043, kmaped@skku.edu

Received 25 July 2014 Revised 15 September 2014 Accepted 4 October 2014

(open-access):

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/).

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.

Keywords: Pulmonary alveolar proteinosis, Child

Figures and Tables

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.

References

1. Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. N Engl J Med. 2003; 349:2527–2539.

2. Carey B, Trapnell BC. The molecular basis of pulmonary alveolar proteinosis. Clin Immunol. 2010; 135:223–235.

3. Froudarakis ME, Koutsopoulos A, Mihailidou HP. Total lung lavage by awake flexible fiberoptic bronchoscope in a 13-year-old girl with pulmonary alveolar proteinosis. Respir Med. 2007; 101:366–369.

4. Byun MK, Kim DS, Kim YW, Chung MP, Shim JJ, Cha SI, et al. Clinical features and outcomes of idiopathic pulmonary alveolar proteinosis in Korean population. J Korean Med Sci. 2010; 25:393–398.

5. Suzuki T, Sakagami T, Rubin BK, Nogee LM, Wood RE, Zimmerman SL, et al. Familial pulmonary alveolar proteinosis caused by mutations in CSF2RA. J Exp Med. 2008; 205:2703–2710.

6. Woo DH, Park JE, Ryu YH, Kim HJ, Shin KC, Chung JH, et al. A case of pulmonary alveolar proteinosis. Yeungnam Univ J Med. 2010; 27:57–62.

7. Rosen SH, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N Engl J Med. 1958; 258:1123–1142.

8. Stanley E, Lieschke GJ, Grail D, Metcalf D, Hodgson G, Gall JA, et al. Granulocyte/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology. Proc Natl Acad Sci U S A. 1994; 91:5592–5596.

9. Kitamura T, Tanaka N, Watanabe J, Uchida , Kanegasaki S, Yamada Y, et al. Idiopathic pulmonary alveolar proteinosis as an autoimmune disease with neutralizing antibody against granulocyte/macrophage colony-stimulating factor. J Exp Med. 1999; 190:875–880.

10. Suzuki T, Sakagami T, Young LR, Carey BC, Wood RE, Luisetti M, et al. Hereditary pulmonary alveolar proteinosis: pathogenesis, presentation, diagnosis, and therapy. Am J Respir Crit Care Med. 2010; 182:1292–1304.

11. Ceruti M, Rodi G, Stella GM, Adami A, Bolongaro A, Baritussio A, et al. Successful whole lung lavage in pulmonary alveolar proteinosis secondary to lysinuric protein intolerance: a case report. Orphanet J Rare Dis. 2007; 2:14.

12. Wang BM, Stern EJ, Schmidt RA, Pierson DJ. Diagnosing pulmonary alveolar proteinosis. A review and an update. Chest. 1997; 111:460–466.

13. Seymour JF, Presneill JJ, Schoch OD, Downie GH, Moore PE, Doyle IR, et al. Therapeutic efficacy of granulocyte-macrophage colony-stimulating factor in patients with idiopathic acquired alveolar proteinosis. Am J Respir Crit Care Med. 2001; 163:524–531.

14. Song JW, Park SH, Kang KW. A case of idiopathic pulmonary alveolar proteinosis treated with granulocyte-macrophage colony stimulating factor (GM-CSF) after partial response to whole lung lavage. Tuberc Respir Dis. 2009; 67:569–573.

15. Tazawa R, Trapnell BC, Inoue Y, Arai T, Takada T, Nasuhara Y, et al. Inhaled granulocyte/macrophage-colony stimulating factor as therapy for pulmonary alveolar proteinosis. Am J Respir Crit Care Med. 2010; 181:1345–1354.

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