Pediatric Gastroenterology, Hepatology & Nutrition

  • 제18권3호
  • /
  • Pages.175-179
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  • 2015
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  • 2234-8646(pISSN)
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  • 2234-8840(eISSN)
대한소아소화기영양학회 (The Korean Society of Pediatric Gastroenterology, Hepatology and Nutrition)
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Outcome of Alagille Syndrome Patients Who Had Previously Received Kasai Operation during Infancy: A Single Center Study

  • Lee, Hwa Pyung (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Kang, Ben (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Choi, So Yoon (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Sanghoon (Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Lee, Suk-Koo (Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine) ;
  • Choe, Yon Ho (Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine)
  • 투고 : 2015.02.05
  • 심사 : 2015.07.21
  • 발행 : 2015.09.30
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초록

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 prognosis and mortality between those who had undergone Kasai operation during infancy (Kasai group) and those who had not (non-Kasai group). Results: Among 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). Conclusion: Although 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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참고문헌

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피인용 문헌

  1. Initial assessment of the infant with neonatal cholestasis—Is this biliary atresia? vol.12, pp.5, 2017, https://doi.org/10.1371/journal.pone.0176275
  2. Alagille Syndrome Mimicking Biliary Atresia Confirmed by Jagged1 (JAG1) Gene Analysis in a Newborn: A Case Report vol.24, pp.3, 2015, https://doi.org/10.5385/nm.2017.24.3.140
  3. Imaging findings of Alagille syndrome in young infants: differentiation from biliary atresia vol.90, pp.1077, 2015, https://doi.org/10.1259/bjr.20170406
  4. Rapid whole-genome sequencing decreases infant morbidity and cost of hospitalization vol.3, pp.None, 2015, https://doi.org/10.1038/s41525-018-0049-4
  5. A Novel c.91dupG JAG1 Gene Mutation Is Associated with Early Onset and Severe Alagille Syndrome vol.2018, pp.None, 2018, https://doi.org/10.1155/2018/1369413
  6. Cholangiopathies – Towards a molecular understanding vol.35, pp.None, 2018, https://doi.org/10.1016/j.ebiom.2018.08.024
  7. Alagille Syndrome: A Novel Mutation in JAG1 Gene vol.7, pp.None, 2015, https://doi.org/10.3389/fped.2019.00199
  8. Outcomes of liver transplantation for Alagille syndrome after Kasai portoenterostomy: Alagille Syndrome with agenesis of extrahepatic bile ducts at porta hepatis vol.54, pp.11, 2019, https://doi.org/10.1016/j.jpedsurg.2019.04.022
  9. Value of the International Classification of Diseases code for identifying children with biliary atresia vol.64, pp.2, 2015, https://doi.org/10.3345/cep.2020.00423
  10. Diagnostic approach to neonatal and infantile cholestasis: A position paper by the SIGENP liver disease working group vol.54, pp.1, 2015, https://doi.org/10.1016/j.dld.2021.09.011