Outcome of Alagille Syndrome Patients Who Had Previously Received Kasai Operation during Infancy: A Single Center Study
2
조회
0
다운로드
2
인용
0
참고문헌
키워드
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 pa-tients 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.
Abstract
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 pa-tients 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.
참고문헌 (13)
- Vajro P, "Alagille syndrome: an overview", Clin Res Hepatol Gastroenterol, 36, pp.275~277 (2012)
- Alagille D, "Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases", J Pediatr, 110, pp.195~200 (1987)
- Kaye AJ, "Effect of Kasai procedure on hepatic outcome in Alagille syndrome", J Pediatr Gastroenterol Nutr, 51, pp.319~321 (2010)
- Turnpenny PD, "Alagille syndrome: pathogenesis, diagnosis and management", Eur J Hum Genet, 20, pp.251~257 (2012)
- Deprettere A, "Syndromic paucity of the intrahepatic bile ducts: diagnostic difficulty;severe morbidity throughout early childhood", J Pediatr Gastroenterol Nutr, 6, pp.865~871 (1987)
- Hoffenberg EJ, "Outcome of syndromic paucity of interlobular bile ducts (Alagille syndrome) with onset of cholestasis in infancy", J Pediatr, 127, pp.220~224 (1995)
- Emerick KM, "Features of Alagille syndrome in 92 patients: frequency and relation to prognosis", Hepatology, 29, pp.822~829 (1999)
- Quiros-Tejeira RE, "Variable morbidity in alagille syndrome: a review of 43 cases", J Pediatr Gastroenterol Nutr, 29, pp.431~437 (1999)
- Subramaniam P, "Diagnosis of Alagille syndrome-25 years of experience at King's College Hospital", J Pediatr Gastroenterol Nutr, 52, pp.84~89 (2011)
- 안경진, "Alagille syndrome and a JAG1 mutation: 41 cases of experience at a single center", Korean Journal of Pediatrics, 58(10), pp.392~397 (2015), 대한소아과학회
- Ganschow R, "Liver transplantation in children with Alagille syndrome", Transplant Proc, 33, pp.3608~3609 (2001)
- Kasahara M, "Living-related liver transplantation for Alagille syndrome", Transplantation, 75, pp.2147~2150 (2003)
- Lykavieris P, "Outcome of liver disease in children with Alagille syndrome:a study of 163 patients", Gut, 49, pp.431~435 (2001)