Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Analysis of reports on orphan lung diseases in Korean children

  • Jang, Sun-Jung (Department of Pediatrics, CHA Bundang Medical Center, CHA University) ;
  • Seo, Hyun-Kyung (Department of Pediatrics, CHA Bundang Medical Center, CHA University) ;
  • Yi, Sung-Jae (Department of Pediatrics, CHA Bundang Medical Center, CHA University) ;
  • Kim, Kyong-Min (Department of Pediatrics, CHA Bundang Medical Center, CHA University) ;
  • Jee, Hye-Mi (Department of Pediatrics, CHA Bundang Medical Center, CHA University) ;
  • Han, Man-Yong (Department of Pediatrics, CHA Bundang Medical Center, CHA University)
  • Received : 2009.09.15
  • Accepted : 2009.10.19
  • Published : 2010.06.15
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Abstract

Purpose: Orphan lung diseases are defined as lung diseases with a prevalence of 1 or less in 2,000 individuals. Despite an increase in the numbers of patients with such diseases, few studies on Korean children have appeared. To obtain epidemiologic and demographic data on these diseases, we systematically reviewed reports on pediatric orphan lung diseases in Korea over the last 50 years. Methods: We reviewed 223 articles that have appeared since 1958 on orphan lung diseases in Korean children. These articles described a total of 519 patients aged between 0 and 18 years. We classified patients by year of publication, diagnosis, geographic region, and journal. Results: Of 519 patients, 401 had congenital cystic lung diseases and 66 had bronchiolitis obliterans. About 80% of patients were described in reports published in three journals, Pediatric Allergy and Respiratory Disease (Korea), the Korean Journal of Pediatrics, and the Korean Journal of Thoracic and Cardiovascular Surgery, in which papers on 157 (30.2%), 138 (26.6%), and 111 (21.4%) patients appeared, respectively. The frequency of publication of case reports has increased since 1990. Of the 519 patients, 401 (77.3%) were from Seoul/Gyeonggi-do and 72 (13.9%) from Busan/Gyeongsangnam-do. Conclusion: The prevalence of pediatric orphan lung disease has increased since 1990, and some provinces of Korea have a higher incidence of these diseases than do others. Studies exploring the incidence of pediatric orphan lung diseases in Korea are needed for effective disease management.

Keywords

References

  1. Shanti CM, Klein MD. Cystic lung disease. Semin Pediatr Surg 2008;7:2-8.
  2. Jaffe A. Orphan lung diseases in childhood: still unadopted? Thorax 2005;60:892-4. https://doi.org/10.1136/thx.2005.050187
  3. Eurordis. Rare diseases: understanding this public health priority. 2005. Available at http://www.eurordis.org/IMG/pdf/princeps_document-EN.pdf [accessed on 14 aug 2009]
  4. Park JS, Pyun BY, Kim YT. A case of idiopathic pulmonary hemosiderosis. Pediatr Allergy Respir Dis(Korea) 1999;9:226-32.
  5. Ellerman A, Bisgaard H. Longitudinal study of lung function in a cohort of primary ciliary dyskinesia. Eur Respir J 1997;10:2376-9. https://doi.org/10.1183/09031936.97.10102376
  6. Kim BJ, Kim HA, Song YH, Yu Jh, Kim Sg, Park SJ, et al. Nationwide surveillance of acute interstitial pneumonia in Korea. Korean Journal of Pediatrics 2008;52:324-9. https://doi.org/10.3345/kjp.2009.52.3.324
  7. Clement A. Task force on chronic interstitial lung disease in immunocompetent children. Eur Respir J 2004; 24:686-97. https://doi.org/10.1183/09031936.04.00089803
  8. Ahn YM, Choi HR, Lee HJ, Dong ES. A case of congenital central hypoventilation syndrome(ondine's curse) with hirschsprung's disease. Pediatr Allergy Respir Dis(Korea) 1993; 3:113-20.
  9. Yu HJ, Nam SW. A case of congenital central hypoventilation syndrome. Korean Journal of Pediatrics Neurol 1996;4:185-92.
  10. Kim YJ, Lee JH, Lee SJ, Lee K. A case of idiopathic pulmonary hemosiderosis. J Korean Pediatr Soc 1985;28:72-6.
  11. Moon HR, Ko TS, Ko YY, Choi JH, Kim YC. Cystic fibrosis: a case presented with recurrent bronchiolitis in infancy in a Korean male infant. J Korean Med Sci 1988;3:157-62.
  12. Kim YS, You JH, Rhee KS, Rha KS. A case of bronchial cast. J Korean Pediatr Soc 1988;31:1232-6.
  13. Ahn GH, Kim YI, Kim SI, Lee SK, Yun HS, Mok JE. Congenital cystic lung - an autopsy case report of a congenital cystic lung associated with hydramnios. Korean J Pathol 1969;3:21-6.
  14. Nam CH, Ahn WS, Hur Y, Park HS, Lee JH, Yu HS. Pulmonary sequestration - report of 2 cases. Korean J Thorac Cardiovasc Surg 1981;14:350-3.
  15. Ko YY, Ko KW, Chi JG. Clinical studies on interstitial pneumonitis in children. J Korean Pediatr Soc 1985; 28:663-73.
  16. Kim TW, Lym DH, Kim JH, Son BK, Han HS, Shin YK. A case of nonspecific interstitial pneumonia in a child. J Korean Pediatr Soc 2002;45:529-34.
  17. Jang HO, Ham SC, Kim YK, Oh SW, Park YW. A case of chronic pneumonitis of infancy in pediatric interstitial lung disease. J Korean Pediatr Soc 2001;44:1085-90.
  18. Lee HY, Kim HM, Nam MK, Kim JS, Jung SH, Kim DJ. A case of lymphocytic interstitial pneumonia. J Korean Pediatr Soc 1997;40:572-7.
  19. Kim SA, Bae SY, Lee SY, Jeong DC, Chung SY, Kang JH. A case of pulmonary sarcoidosis in a 6-year-old girl. Pediatr Allergy Respir Dis(Korea) 2006;16:253-8.
  20. Shim JW, Lee DK. A case of chronic eosinophilic pneumonia confusing as foreign body aspiration. Pediatr Allergy Respir Dis(Korea) 1995;5:168-74.
  21. Du Bois RM. Rare lung diseases: orphans no more? Am J Respir Crit Care Med 2002;166:1157-8. https://doi.org/10.1164/rccm.2208002
  22. Hong T, Michelle AF, Joe C, Fiona C. Congenital cystic adenomatoid malformation: Monitoring the antenatal and short-term neonatal outcome. J Aust N Z J Obstet Gynaecol 2008; 48:462-6. https://doi.org/10.1111/j.1479-828X.2008.00887.x
  23. Lee HJ, Sun GG, Na KH, Park SY, Kim EY, Kim KS, et al. Three cases of pulmonary paragonimiasis in a family after Ingestion of raw freshwater crayfishes caught in a stream of Wolchulmountain. Korean Journal of Pediatrics Infec Dis 2002; 9:222-9.
  24. Jeon KM, Koh WJ, Kim HJ, Kwon OJ, Kim TS, Lee KS, et al. Clinical features of recently diagnosed pulmonary paragonimiasis in Korea. Chest 2005;28:1423-30.
  25. Chung JH, Ha SJ, Kim BS, Hong SJ. Clinical spectrum and lung pathology in children with interstitial lung disease. J Korean Assoc Pediatr Surg 2002;45:79-87.
  26. Lee SJ, Shin EW, Park EY, Oh PS, Lee KH, Kim KN, et al. A case of idiopathic interstitial pneumonia in childhood. Korean Journal of Pediatrics 2005;48:327-32.
  27. Yamashiro Y, Shimizu T, Oguchi S, Shioya T, Nagata S, Ohtsuka Y. The estimated incidence of cystic fibrosis in Japan. J Pediatr Gastroenterol Nutr 1997;24:544-7. https://doi.org/10.1097/00005176-199705000-00010

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