2017
DOI: 10.1097/md.0000000000007267
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Surgical outcome and etiologic heterogeneity of infants with biliary atresia who received Kasai operation less than 60 days after birth

Abstract: This study aimed to analyze the impact of etiologic heterogeneity and operation age on prognosis of infants with biliary atresia (BA) who received Kasai operation prior to 60 days of age.From 2004 to 2010, 158 infants received Kasai operation before turning 60 days old. According to Davenport 2012 classifications, 4 groups of BA were defined: cystic BA, syndrome BA, and associated malformation, cytomegalovirus (CMV)-associated BA, and isolated BA. Native (autologous) liver survival rates and incidence of chola… Show more

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Cited by 36 publications
(23 citation statements)
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“…[3][4][5][6][7][8] Several studies have proposed that early diagnosis and treatment is key to the restoration of bile flow and favorable prognosis. [9][10][11][12][13] However, many other non-BA cholestatic diseases, such as idiopathic cholestasis, Alagille syndrome, and progressive familial intrahepatic cholestasis, are similar to BA in symptoms and noninvasive laboratory and radiologic test results, leading to difficulties in the accurate diagnosis of BA. Our previous retrospective study showed that 14% of 602 patients with BA-like neonatal jaundice were found to be non-BA by using invasive diagnostic procedures such as intraoperative cholangiography and liver biopsy.…”
Section: What This Study Addsmentioning
confidence: 99%
“…[3][4][5][6][7][8] Several studies have proposed that early diagnosis and treatment is key to the restoration of bile flow and favorable prognosis. [9][10][11][12][13] However, many other non-BA cholestatic diseases, such as idiopathic cholestasis, Alagille syndrome, and progressive familial intrahepatic cholestasis, are similar to BA in symptoms and noninvasive laboratory and radiologic test results, leading to difficulties in the accurate diagnosis of BA. Our previous retrospective study showed that 14% of 602 patients with BA-like neonatal jaundice were found to be non-BA by using invasive diagnostic procedures such as intraoperative cholangiography and liver biopsy.…”
Section: What This Study Addsmentioning
confidence: 99%
“…The prevalence of BA in the Chinese population is higher comparing to the Caucasian population [9]. At the same time, the rate of cytomegalovirus infection in Chinese population is also higher than in western countries.…”
Section: Discussionmentioning
confidence: 81%
“…A young age, especially <60 days old at KPE, has been widely, although not universally, linked to improved outcome in BA, which may be explained by the milder liver fibrosis in younger patients ( 16 , 39 41 ). However, Song et al ( 42 ) demonstrated that BA patients younger than 40 days had a poorer NLS rate and that, in these patients, severe liver fibrosis may account for the reverse consequence ( 35 , 39 ). Interestingly, our results showed that age at KPE had no significant impact on prognosis of BA.…”
Section: Discussionmentioning
confidence: 99%