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Efficacy of prostaglandin E2 and ursodeoxycholic acid after portoenterostomy in the prognostically unfavorable type III biliary atresia

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Abstract

Nineteen patients with type III congenital biliary atresia (CBA) were treated by portoenterostomy. In the histological sections, bile ductule diameters below 100 μm and age-dependent liver fibrosis of grade II–IV were found. In group A, 8 patients received no postoperative choleretic therapy; in group B, 6 patients were treated with prostaglandin E2 (PG E2); and in group C, in 5 patients ursodeoxycholic acid (UDCA) was added to the PG E2 therapy. In the first 3 postoperative months a significant beneficial effect of PG E2 was observed as reduced serum bilirubin and aspartate aminotransferase (AST) levels relative to the untreated group. In group C (UDCA and PG E2) significantly lower serum levels of all liver parameters relative to the untreated group and gradually decreased levels of bilirubin, alanine aminotransferase (ALT), g-glutamyl transferase (GGT), and higher cholinesterase (CHE) activity were observed than in the PG E2 group. During episodes of cholangitis steroids were also added, but alterations in serum bilirubin levels, CHE activity, and bile flow did not improve after any adjunctive therapy. Liver enzyme activity was immediately elevated in groups A and B, but significantly lower in group C. The efficacy of both drugs, PG E2 and UDCA, lasted only for the first 3 post-operative months and failed to improve or delay the progression of hepatic damage. After portoenterostomy, 13 patients (68%), died within 6–32 months, 12 from progressive liver failure and 1 after liver transplantation. Six patients (32%) are alive, 3 after liver transplantation; 2 are jaundice-free and 1 jaundiced patient is on a waiting list for transplantation. In these prognostically poor patients with type III CBA, early liver transplantation is the only feasible option as adjuvant Pg E2, UDCA, and corticosteroid therapy demonstrated no long-term improvement after portoenterostomy.

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Authors and Affiliations

  1. Department of Pediatric Surgery, University of Graz, Medical School, Heinrichstrasse 31, A-8010, Graz, Austria

    G. Schimpl, H. Sauer & P. H. Schober

  2. Department of Pediatrics, University of Graz, Medical School, Heinrichsrasse 31, A-8010, Graz, Austai

    J. Deutsch

  3. Department of Pathology, University of Graz, Medical School, Heinrichstrasse 31, A-8010, Graz, Austria

    H. Becker

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  1. G. Schimpl
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  2. H. Sauer
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  4. P. H. Schober
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Correspondence to: G. Schimpl

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Schimpl, G., Sauer, H., Deutsch, J. et al. Efficacy of prostaglandin E2 and ursodeoxycholic acid after portoenterostomy in the prognostically unfavorable type III biliary atresia. Pediatr Surg Int 7, 438–443 (1992). https://doi.org/10.1007/BF00178824

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