Top

Pediatric Surgery International

Published in:

01-10-2018 | Original Article

Outcomes of Alagille syndrome following the Kasai operation: a systematic review and meta-analysis

Authors: Jun Fujishiro, Kan Suzuki, Miho Watanabe, Chizue Uotani, Toshiko Takezoe, Naohiro Takamoto, Kentaro Hayashi

Published in: Pediatric Surgery International | Issue 10/2018

Abstract

Purpose

Infants with Alagille syndrome (AGS) frequently develop neonatal cholestasis, and some AGS infants who suspected of biliary atresia subsequently undergo the Kasai operation with the diagnosis of biliary atresia. The aim of this study was to investigate the effect of the Kasai operation on liver and patient outcomes among AGS patients, using a meta-analysis.

Methods

A systematic review and meta-analysis of studies describing the outcomes of AGS patients with/without the Kasai operation were conducted. The analyzed outcomes were liver transplantation, not living with the native liver, and mortality for any reason.

Results

We identified 6 studies (394 AGS patients). All studies were retrospective cohort or case-control studies. The incidences of liver transplantation, not living with the native liver, and mortality were significantly higher in AGS patients who underwent the Kasai operation than in those who did not undergo the Kasai operation (odds ratio: 6.46, 95% CI 3.23–12.89, p < 0.00001; odds ratio: 25.88, 95% CI 2.83–236.84, p < 0.004; odds ratio: 15.05, 95% CI 2.70–83.93, p = 0.002, respectively).

Conclusion

The Kasai operation was associated with poor outcomes in AGS patients. It remains unclear if the Kasai operation directly deteriorates liver and patient outcomes in AGS patients.

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

Danks DM, Campbell PE, Jack I, Rogers J, Smith AL (1977) Studies of the aetiology of neonatal hepatitis and biliary atresia. Arch Dis Child 52:360–367CrossRefPubMedPubMedCentral

Turnpenny PD, Ellard S (2012) Alagille syndrome: pathogenesis, diagnosis and management. Eur J Hum Genet EJHG 20:251–257. https://doi.org/10.1038/ejhg.2011.181 CrossRefPubMed

Kamath BM, Bauer RC, Loomes KM, Chao G, Gerfen J, Hutchinson A, Hardikar W, Hirschfield G, Jara P, Krantz ID, Lapunzina P, Leonard L, Ling S, Ng VL, Hoang PL, Piccoli DA, Spinner NB (2012) NOTCH2 mutations in Alagille syndrome. J Med Genet 49:138–144CrossRefPubMed

Li L, Krantz ID, Deng Y, Genin A, Banta AB, Collins CC, Qi M, Trask BJ, Kuo WL, Cochran J, Costa T, Pierpont ME, Rand EB, Piccoli DA, Hood L, Spinner NB (1997) Alagille syndrome is caused by mutations in human Jagged1, which encodes a ligand for Notch1. Nat Genet 16:243–251. https://doi.org/10.1038/ng0797-243 CrossRefPubMed

Oda T, Elkahloun AG, Pike BL, Okajima K, Krantz ID, Genin A, Piccoli DA, Meltzer PS, Spinner NB, Collins FS, Chandrasekharappa SC (1997) Mutations in the human Jagged1 gene are responsible for Alagille syndrome. Nat Genet 16:235–242. https://doi.org/10.1038/ng0797-235 CrossRefPubMed

McDaniell R, Warthen DM, Sanchez-Lara PA, Pai A, Krantz ID, Piccoli DA, Spinner NB (2006) NOTCH2 mutations cause Alagille syndrome, a heterogeneous disorder of the notch signaling pathway. Am J Hum Genet 79:169–173. https://doi.org/10.1086/505332 CrossRefPubMedPubMedCentral

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

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

10.

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

11.

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

12.

Lee HP, Kang B, Choi SY, Lee S, Lee SK, Choe YH (2015) Outcome of alagille syndrome patients who had previously received kasai operation during infancy: a single center study. Pediatr Gastroenterol Hepatol Nutr 18:175–179CrossRefPubMedPubMedCentral

13.

Quiros-Tejeira RE, Ament ME, Heyman MB, Martin MG, Rosenthal P, Hall TR, McDiarmid SV, Vargas JH (1999) Variable morbidity in alagille syndrome: a review of 43 cases. J Pediatr Gastroenterol Nutr 29:431–437CrossRefPubMed

14.

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

15.

Dedic T, Jirsa M, Keil R, Rygl M, Snajdauf J, Kotalova R (2015) Alagille syndrome mimicking biliary atresia in early infancy. PLoS One 10:e0143939CrossRefPubMedPubMedCentral

16.

Subramaniam P, Knisely A, Portmann B, Qureshi SA, Aclimandos WA, Karani JB, Baker AJ (2011) Diagnosis of alagille syndrome-25 years of experience at King’s College Hospital. J Pediatr Gastroenterol Nutr 52:84–89CrossRefPubMed

Title: Outcomes of Alagille syndrome following the Kasai operation: a systematic review and meta-analysis
Authors: Jun Fujishiro
Kan Suzuki
Miho Watanabe
Chizue Uotani
Toshiko Takezoe
Naohiro Takamoto
Kentaro Hayashi
Publication date: 01-10-2018
Publisher: Springer Berlin Heidelberg
Published in: Pediatric Surgery International / Issue 10/2018
Print ISSN: 0179-0358
Electronic ISSN: 1437-9813
DOI: https://doi.org/10.1007/s00383-018-4316-3

At a glance: The STEP trials

Springer Medicine

Outcomes of Alagille syndrome following the Kasai operation: a systematic review and meta-analysis

Abstract

Purpose

Methods

Results

Conclusion

At a glance: The STEP trials

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 10/2018

Introduction of resection of intrahepatic bile duct stenosis-causing membrane or septum into laparoscopic choledochal cyst excision

Pulmonary function in children with Pectus excavatum and post-operative changes after nuss procedure

Clinical implications of serum Mac-2-binding protein (M2BPGi) during regular follow-up of patients with biliary atresia

Neonates undergoing gastrointestinal surgery have a higher incidence of non-IgE-mediated gastrointestinal food allergies

Predictive factors affecting the prognosis and late complications of 73 consecutive cases of esophageal atresia at 2 centers

Pitfalls in the management of congenital tracheal stenosis: is conservative management feasible?