Outcome of Alagille Syndrome Patients Who Had Previously Received Kasai Operation during Infancy: A Single Center Study

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Outcome of Alagille Syndrome Patients Who Had Previously Received Kasai Operation during Infancy: A Single Center Study

PURPOSE Infants with Alagille syndrome (AGS) are occasionally misdiagnosed as biliary atresia and subsequently undergo Kasai operation. The purpose of this study was to investigate the outcome of patients with AGS who had previously received Kasai operation during infancy. METHODS This retrospective study was conducted at the Department of Pediatrics, Samsung Medical Center. We compared the p...

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Surgical outcome and etiologic heterogeneity of infants with biliary atresia who received Kasai operation less than 60 days after birth

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,...

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Alagille syndrome with a previously undescribed mutation.

BACKGROUND Alagille Syndrome is a rare genetic disease characterized by abnormalities of the intrahepatic biliary ducts with cholestasis along with multisystem anomalies. CASE CHARACTERISTICS An 8-year old child with persisting jaundice, severe itching and failure to thrive. OBSERVATION Diagnosis of Alagille syndrome was made on the basis of clinical features, typical facies and liver biops...

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ژورنال

عنوان ژورنال: Pediatric Gastroenterology, Hepatology & Nutrition

سال: 2015

ISSN: 2234-8646,2234-8840

DOI: 10.5223/pghn.2015.18.3.175