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Prognosis of Biliary Atresia After 2-year Survival With Native Liver

A Nationwide Cohort Analysis

Witt, Mauri*; van Wessel, Daan B.E.*; de Kleine, Ruben H.J.; Bruggink, Janneke L.M.*; Hulscher, Jan B.F.*; Verkade, Henkjan J. On behalf of NeSBAR (Netherlands Study group on Biliary Atresia Registry)

Journal of Pediatric Gastroenterology and Nutrition: December 2018 - Volume 67 - Issue 6 - p 689–694
doi: 10.1097/MPG.0000000000002130
Original Articles: Hepatology

Objectives: The aim of the study is to determine the prognosis of patients with biliary atresia after 2 years of native liver survival (NLS) and to identify prognostic factors for continued NLS after 2 years of age.

Methods: We retrospectively analyzed perioperative, laboratory, and outcome parameters of all biliary atresia patients in The Netherlands between January 1987 and June 2015 with NLS of at least 2 years. We compared parameters between patients who continued to have their native liver (NLS+) to those who did not, either by transplant or death (NLS−).

Results: We included 100 patients. Upon a median follow-up of 16.4 years, NLS ended in 37% by liver transplantation (LTx) and in 6% by (pre-transplant) mortality. NLS rates at 5, 10, 15, 18 years of age were 89%, 72%, 60%, 54%, respectively. Corresponding overall survival rates were 98%, 90%, 87%, 87%, respectively. Six months post-Kasai, NLS+ patients had higher clearance of jaundice (COJ) rate, significantly lower total and direct serum bilirubin, aspartate-aminotransferase and alkaline phosphatase levels, compared with NLS− patients (each P < 0.05). Cox regression could only assess a significant effect of COJ on continued NLS. Main indications for LTx after the age of 2 were irreversible jaundice and portal hypertension.

Conclusions: Eighty-seven percent of patients with 2-year NLS reach adult age and more than 50% with their native liver. A pre-transplant mortality of 6%, however, exists among patients who reach the age of 2 years with their native livers. Early life parameters, other than COJ, did not have a significant effect on continued NLS after 2 years of age.

*Department of Pediatric Surgery

Department of Hepato-Pancreato-Biliary Surgery

Department of Pediatric Gastroenterology, Hepatology and Nutrition, University Medical Center Groningen, Groningen, The Netherlands.

Address correspondence and reprint requests to Mauri Witt, MD, Department of Pediatric Surgery University Medical Center Groningen, PO box 30.001, 9700 RB Groningen, The Netherlands (e-mail: m.witt@umcg.nl).

Received 9 May, 2018

Accepted 30 July, 2018

Mauri Witt and Daan B.E. van Wessel contributed equally to this work.

The authors report no conflicts of interest.

© 2018 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,