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- W2085284611 abstract "Introduction: Biliary atresia (BA) is a neonatal obstructive cholangiopathy characterized by a fibrosclerosing obliteration of the extrahepatic bile duct. Even with early surgery, most patients (70–80%) develop end-stage biliary cirrhosis and require liver transplantation. Portal hypertension has been documented in 68% of these children with its consequences, like massive bleeding from rupture of esophageal or gastric varices. Objective: Characterizing the clinical outcome to portal hypertension of BA with drainage after Kasai procedure (Conjugated Bilirrubin <2mg/dl). Methods: 15 children with BA with drainage after Kasai procedure (Conjugated Bilirrubin <2mg/dl)attended at our institution from 1986 through 2003 were selected. We reviewed the charts for clinical history, physical findings, laboratory values and endoscopic study. Clinical characteristics (gender, age of surgery, onset of splenomegaly, ascites, esophageal or gastric varices, bleeding) and biochemical characteristics (platelet count) at 6, 12, 18 and 24 months were described. Results: The mean age at the time of Kasai procedure was 9,45w. The splenomegaly was first detected with a median age of 0.48y (0.37–1.45; n=14). The onset of plaquetopenia had a median age of 2.57y (0.91–6.13; n=13). Small varices started with a median age of 1,34y (0.96–1.47; n=8); medium varices appeared at a median age of 2,40 y (1.01–5.05; n=8); large varices appeared at median age of 2.87 y (2.42–6.43; n=6), and red cherry spots was detected with a median age of 2.71 (2.03–4.31; n=5). The first bleeding occurred at mean age of 2.98y (n=4). Ascite appeared with a median age of 1.81y (0.73–4.77; n=5). Conclusion: Children with drainage after Kasai procedure develop portal hypertension with esophageal varices needing endoscopic evaluation in the first year of life to introduce prophylactic therapy because they have the probability to present bleeding at early stages of life (at about 3 years old)." @default.
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- W2085284611 date "2004-06-01" @default.
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- W2085284611 title "P0101 PP PORTAL HYPERTENSION IN BILIARY ATRESIA WITH DRAINAGE AFTER KASAI PROCEDURE" @default.
- W2085284611 doi "https://doi.org/10.1097/00005176-200406001-00225" @default.
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