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KMID : 0605920130190010001
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Journal of Korean Association of Pediatric Surgeons
2013 Volume.19 No. 1 p.1 ~ p.13
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Biliary Atresia -A Survey by the Korean Association of Pediatric Surgeons in 2011-
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Oh Jung-Tak
Kim Dae-Yeon
Kim Seong-Cheol
Kim In-Gu
Kim Hae-Yeong
Kim Hyun-Yung
Nam So-Hyun
Park Kwi-Won
Park Woo-Hyun
Park Jin-Young
Seo Jeong-Min
Lee Nam-Hyuk
Lee Myung-Duk
Lee Suk-Koo
Lee Sung-Cheol
Jung Sang-Young
Jung Sung-Eun
Chung Jae-Hee
Choi Kum-Ja
Chio Soon-Ok
Choi Seung-Hoon
Choi Yun-Mee
Han Seok-Joo
Hong Jeong
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Abstract
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The Korean Association of Pediatric Surgeons (KAPS) performed the second nationwide survey on biliary atresia in 2011. It was a follow-up study to the first survey, which was performed in 2001 for the retrospective analysis of biliary
atresia between 1980 and 2000. In the second survey, the authors reviewed and analyzed the clinical data of patients who were treated for biliary atresia by the members of KAPS from 2001 to 2010. A total of 459 patients were registered. Among
them, 435 patients primarily underwent the Kasai operation. The mean age of patients who underwent the Kasai operation was 66.2±28.7 days, and 89.7% of those patients had type III biliary atresia. Only five patients (1.4%) had complications
related to the Kasai operation. After the Kasai operation, 269 (61.8%) of the patients were re-admitted because of cholangitis (79.9%) and varices (20.4%). One hundred and fifty-nine (36.6%) of the patients who underwent the Kasai operation subsequently underwent liver transplantation. The most common cause of subsequent liver transplantation was persistent hyperbilirubinemia. The mean interval between the Kasai operation and liver transplantation was 1.1±1.3 years. Overall the 10-year survival rate after the Kasai operation was 92.9% and the 10-year native liver survival rate was 59.8%. We had 23 patients for primary liver transplantation without the Kasai operation. The mean age patients who underwent primary liver transplantation was 8.6± 2.9 months. In summary, among the 458 Kasai-operation and liver-transplantation patients, 373 lived, 31 died, and 54 were unavailable for follow up. One-third of the patient who survived have had complications correlated with biliary atresia. In comparison with the first survey, this study showed a higher survival rate and a greater number of liver transplantation.
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KEYWORD
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Biliary atresia, Korea, Kasai operation, Liver transplantation
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