Twenty-year transplant-free survival rate among patients with biliary atresia.

Abstract

BACKGROUND & AIMS:

Surgical treatment with Kasai portoenterostomy has improved the prognosis for patients with biliary atresia, although most patients ultimately require liver transplantation. Well-described patients with long-term, transplant-free survival are scarce; we assessed liver status and health perception among Dutch patients who survived 20 years after therapy and investigated whether the rate of transplant-free survival increases with time.

METHODS:

By using the Dutch national database for biliary atresia, we identified 104 patients, born between 1977 and 1988. We collected data on clinical characteristics, liver biochemistry, and ultrasonography from all transplant-free patients who were alive at age 20 years (n = 28; 27% of the patients). General health perception data (RAND-36) were collected at the last examination.

RESULTS:

The 20-year transplant-free survival rate increased from 20% (10 of 49) in the 1977 to 1982 cohort to 32% (18 of 55) in the 1983 to 1988 cohort (P = .03). Twenty-one percent of the long-term survivors (6 of 28) had normal liver biochemistry test results and no clinical or ultrasonographic signs of cirrhosis. The general health perception of female, but not male, patients, was lower, compared with controls (RAND-36 score, 54 ± 14 vs 74 ± 18; P = .005).

CONCLUSIONS:

More than 25% of patients with biliary atresia survive at least 20 years without liver transplantation in The Netherlands. Women with biliary atresia have a reduced perception of their health, compared with control patients. Twenty percent of long-term survivors are symptom-free, without clinical or ultrasonographic signs of cirrhosis or portal hypertension.

• Authors: Willemien de Vries, Jenneke Homan-Van der Veen, Jan B F Hulscher, Josette E H M Hoekstra-Weebers, Roderick H J Houwen, Henkjan J Verkade, Netherlands Study Group of Biliary Atresia Registry
• Journal: Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association (Clin Gastroenterol Hepatol) Vol. 9 Issue 12 Pg. 1086-91 (Dec 2011) ISSN: 1542-7714 [Electronic] United States
• PMID: 21820397 (Publication Type: Journal Article)
• Copyright: Copyright © 2011 AGA Institute. Published by Elsevier Inc. All rights reserved.

Topics

• Adult
• Biliary Atresia (mortality, psychology, surgery)
• Child
• Child, Preschool
• Female
• Humans
• Infant
• Liver (diagnostic imaging, pathology, physiopathology)
• Liver Function Tests
• Male
• Netherlands
• Survival Analysis
• Ultrasonography