Immunosuppression as adjuvant therapy for biliary atresia

Peter W. Dillon, Elizabeth Owings, Robert Cilley, Douglas Field, Adrian Curnow, Keith Georgeson

Research output: Contribution to journalArticle

73 Citations (Scopus)

Abstract

Background/Purpose: Despite improvements in the surgical management of biliary atresia, the long-term incidence of progressive liver failure remains high. Because chronic inflammation involving both bile ducts and liver parenchyma contributes to the pathology, the authors have hypothesized that the liver damage may be altered using immunosuppressive therapy. The aim of this study was to examine the safety and efficacy of long-term steroid therapy in patients with biliary atresia. Methods: A retrospective analysis of all patients with biliary atresia treated with an hepatoportoenterostomy and postoperative steroid therapy at our 3 institutions was undertaken. Patients were treated uniformly with immunosuppressive doses of oral steroids for a minimum of 6 weeks after surgery. Results: Twenty-five infants with biliary atresia were treated with steroid therapy. Overall survival rate was 22 patients (88%) with a mean follow-up period of 50 months. Nineteen patients (76%) became jaundice free with native liver function. Four patients (16%) did not respond to treatment and required transplantation. Age less than 12 weeks was a crucial predictor of success of adjuvant steroid therapy. Cholangitis developed in 8 patients (32%). There were no complications caused by steroid therapy. Conclusions: Steroid administration at immunosuppressive doses markedly improves the clinical outcome within the first 5 years after surgery as measured by jaundice-free status and survival without liver transplantation when compared with concurrent reports. These results suggest that immunosuppressive therapy is safe and has a positive impact on the clinical course of this disease. However, a randomized study is needed to ultimately prove such an hypothesis.

Original languageEnglish (US)
Pages (from-to)80-85
Number of pages6
JournalJournal of pediatric surgery
Volume36
Issue number1
DOIs
StatePublished - Jan 1 2001

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Biliary Atresia
Immunosuppression
Steroids
Immunosuppressive Agents
Therapeutics
Jaundice
Liver
Cholangitis
Liver Failure
Bile Ducts
Liver Transplantation
Survival Rate
Transplantation
Pathology
Inflammation
Safety
Survival
Incidence

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

Dillon, Peter W. ; Owings, Elizabeth ; Cilley, Robert ; Field, Douglas ; Curnow, Adrian ; Georgeson, Keith. / Immunosuppression as adjuvant therapy for biliary atresia. In: Journal of pediatric surgery. 2001 ; Vol. 36, No. 1. pp. 80-85.
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abstract = "Background/Purpose: Despite improvements in the surgical management of biliary atresia, the long-term incidence of progressive liver failure remains high. Because chronic inflammation involving both bile ducts and liver parenchyma contributes to the pathology, the authors have hypothesized that the liver damage may be altered using immunosuppressive therapy. The aim of this study was to examine the safety and efficacy of long-term steroid therapy in patients with biliary atresia. Methods: A retrospective analysis of all patients with biliary atresia treated with an hepatoportoenterostomy and postoperative steroid therapy at our 3 institutions was undertaken. Patients were treated uniformly with immunosuppressive doses of oral steroids for a minimum of 6 weeks after surgery. Results: Twenty-five infants with biliary atresia were treated with steroid therapy. Overall survival rate was 22 patients (88{\%}) with a mean follow-up period of 50 months. Nineteen patients (76{\%}) became jaundice free with native liver function. Four patients (16{\%}) did not respond to treatment and required transplantation. Age less than 12 weeks was a crucial predictor of success of adjuvant steroid therapy. Cholangitis developed in 8 patients (32{\%}). There were no complications caused by steroid therapy. Conclusions: Steroid administration at immunosuppressive doses markedly improves the clinical outcome within the first 5 years after surgery as measured by jaundice-free status and survival without liver transplantation when compared with concurrent reports. These results suggest that immunosuppressive therapy is safe and has a positive impact on the clinical course of this disease. However, a randomized study is needed to ultimately prove such an hypothesis.",
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Immunosuppression as adjuvant therapy for biliary atresia. / Dillon, Peter W.; Owings, Elizabeth; Cilley, Robert; Field, Douglas; Curnow, Adrian; Georgeson, Keith.

In: Journal of pediatric surgery, Vol. 36, No. 1, 01.01.2001, p. 80-85.

Research output: Contribution to journalArticle

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