Pediatr Gastroenterol Hepatol Nutr.  2015 Sep;18(3):175-179. 10.5223/pghn.2015.18.3.175.

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

• Lee HP
• Kang B
• Choi SY
• Lee S
• Lee SK
• Choe YH

• KMID: 2068794
• DOI: http://doi.org/10.5223/pghn.2015.18.3.175

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 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.

Keyword

Alagille syndrome; Kasai operation; Cholestasis; Liver transplantation

MeSH Terms

Alagille Syndrome*
Biliary Atresia
Child
Cholestasis
Humans
Infant
Liver Transplantation
Mortality
Pediatrics
Prognosis
Retrospective Studies

Reference

1. Vajro P, Ferrante L, Paolella G. Alagille syndrome: an overview. Clin Res Hepatol Gastroenterol. 2012; 36:275–277.

2. Alagille D, Estrada A, Hadchouel M, Gautier M, Odièvre M, Dommergues JP. Syndromic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia): review of 80 cases. J Pediatr. 1987; 110:195–200.

3. Kaye AJ, Rand EB, Munoz PS, Spinner NB, Flake AW, Kamath BM. Effect of Kasai procedure on hepatic outcome in Alagille syndrome. J Pediatr Gastroenterol Nutr. 2010; 51:319–321.

4. Turnpenny PD, Ellard S. Alagille syndrome: pathogenesis, diagnosis and management. Eur J Hum Genet. 2012; 20:251–257.

5. Deprettere A, Portmann B, Mowat AP. Syndromic paucity of the intrahepatic bile ducts: diagnostic difficulty; severe morbidity throughout early childhood. J Pediatr Gastroenterol Nutr. 1987; 6:865–871.

6. Hoffenberg EJ, Narkewicz MR, Sondheimer JM, Smith DJ, Silverman A, Sokol RJ. Outcome of syndromic paucity of interlobular bile ducts (Alagille syndrome) with onset of cholestasis in infancy. J Pediatr. 1995; 127:220–224.

7. Emerick KM, Rand EB, Goldmuntz E, Krantz ID, Spinner NB, Piccoli DA. Features of Alagille syndrome in 92 patients: frequency and relation to prognosis. Hepatology. 1999; 29:822–829.

8. Quiros-Tejeira RE, Ament ME, Heyman MB, Martin MG, Rosenthal P, Hall TR, et al. Variable morbidity in alagille syndrome: a review of 43 cases. J Pediatr Gastroenterol Nutr. 1999; 29:431–437.

9. Subramaniam P, Knisely A, Portmann B, Qureshi SA, Aclimandos WA, Karani JB, et al. Diagnosis of Alagille syndrome-25 years of experience at King's College Hospital. J Pediatr Gastroenterol Nutr. 2011; 52:84–89.

10. Ahn KJ, Bae EJ, Yoon JK, Kim GB, Kwon BS, Moon JS, et al. Alagille syndrome and a JAG1 mutation: 41 cases of experience at a single center. Korean J Pediatr. 2014; [Epub ahead of print].

11. Ganschow R, Grabhorn E, Helmke K, Rogiers X, Burdelski M. Liver transplantation in children with Alagille syndrome. Transplant Proc. 2001; 33:3608–3609.

12. Kasahara M, Kiuchi T, Inomata Y, Uryuhara K, Sakamoto S, Ito T, et al. Living-related liver transplantation for Alagille syndrome. Transplantation. 2003; 75:2147–2150.

13. Lykavieris P, Hadchouel M, Chardot C, Bernard O. Outcome of liver disease in children with Alagille syndrome: a study of 163 patients. Gut. 2001; 49:431–435.