ArticlePediatric gastroenterology, hepatology & nutrition2015
Outcome of Alagille Syndrome Patients Who Had Previously Received Kasai Operation during Infancy: A Single Center Study.
Article in Pediatric gastroenterology, hepatology & nutrition, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers, 1 of them a synthesis that pooled it.
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Who cites it
13 citing papers in PubMed, 1 synthesis or guideline pooled it, 22 citations in OpenAlex.
- Outcomes of Alagille syndrome following the Kasai operation: a systematic review and meta-analysis.Pediatric surgery international · 2018Pooled it
- Trial
- Exome-wide assessment of isolated biliary atresia: A report from the National Birth Defects Prevention Study using child-parent trios and a case-control design to identify novel rare variants.American journal of medical genetics. Part A · 2023Article
- Protocols of Investigation of Neonatal Cholestasis-A Critical Appraisal.Healthcare (Basel, Switzerland) · 2022Review
- Multidisciplinary Management of Alagille Syndrome.Journal of multidisciplinary healthcare · 2022Review
- Re-evaluation of Laparoscopic Hepatic Subcapsular Spider-Like Telangiectasis Sign: A Highly Accurate Method to Diagnose Biliary Atresia in Infants.Frontiers in pediatrics · 2022Article
- Value of the International Classification of Diseases code for identifying children with biliary atresia.Clinical and experimental pediatrics · 2021Article
- Alagille Syndrome: A Novel Mutation inFrontiers in pediatrics · 2019Article
- Cholangiopathies - Towards a molecular understanding.EBioMedicine · 2018Review
- Rapid whole-genome sequencing decreases infant morbidity and cost of hospitalization.NPJ genomic medicine · 2018Article
- A Novel c.91dupGCase reports in genetics · 2018Article
- Imaging findings of Alagille syndrome in young infants: differentiation from biliary atresia.The British journal of radiology · 2017Article
- Biliary Atresia: advances and challenges in early diagnosis and management.Jornal de pediatriaReview
Corrections and comments
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Authors and funding
6 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
purposeInfants 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.
methodsThis retrospective study was conducted at the Department of Pediatrics, Samsung Medical Center. We compared the prognosis and mortality between those who had undergone Kasai operation during infancy (Kasai group) and those who had not (non-Kasai group).
resultsAmong the 15 children with AGS, five had received Kasai operation, while 10 had not. All subjects in the Kasai group revealed neonatal cholestasis, while 70% of the non-Kasai group showed neonatal cholestasis. Liver transplantation was performed in 100% (5/5) among the Kasai group, and 20.0% (2/10) among the non-Kasai group (p=0.007). Mortality was observed in 60.0% (3/5) among the Kasai group, and 10.0% (1/10) among the non-Kasai group (p=0.077).
conclusionAlthough overall mortality rate did not significantly differ between the two groups, the proportion of patients receiving liver transplantation was significantly higher in the non-Kasai group. The relatively worse outcome in AGS patients who had received Kasai operation may be due to the unfavorable influences of Kasai operation on the clinical course of AGS, or maybe due to neonatal cholestasis, irrespective of the Kasai operation.
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