Efficacy of and prognosis after steroid pulse therapy in patients with poor reduction of jaundice after laparoscopic Kasai portoenterostomy

  • Yujiro TanakaEmail author
  • Chiyoe Shirota
  • Takahisa Tainaka
  • Wataru Sumida
  • Kazuo Oshima
  • Satoshi Makita
  • Tomoko Tanaka
  • Yukiko Tani
  • Kosuke Chiba
  • Hiroo Uchida
Original Article



High-dose postoperative steroid therapy after Kasai portoenterostomy is reported to improve jaundice clearance and a strong anti-inflammatory activity might prevent fibrous tissue formation which is often observed at the porta hepatis in revision surgery. We started steroid pulse therapy for the patients with cessation of decrease in jaundice and aimed to evaluate the efficacy in this study.


The demographics and outcomes of patients who underwent laparoscopic Kasai portoenterostomy and received steroid pulse therapy within 2 months postoperatively between September 2014 and December 2018 were retrospectively reviewed; the therapy was determined successful when the serum total bilirubin level decreased to or below two-thirds of the pre-therapy level after 2 weeks. Patient data in the successful group were compared with those in the unsuccessful group.


Steroid pulse therapy was successful in seven of 16 patients (43.8%). The percentage of patients whose serum total bilirubin level decreased to normal was significantly higher in the successful group at 3 months (85.7% vs. 11.1%, P = 0.0028) and after all (100% vs. 33.3%, P = 0.011).


Steroid pulse therapy was effective for some patients. Unsuccessful cases may have little chances of jaundice clearance; revision Kasai portoenterostomy would be a good option.


Biliary atresia Steroid pulse therapy Kasai portoenterostomy Revision surgery Endoscopic surgery 


Compliance with ethical standards

Conflict of interest

The authors declare that they have no competing interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all participants included in this study.


  1. 1.
    Davenport M, Parsons C, Tizzard S, Hadzic N (2013) Steroids in biliary atresia: single surgeon, single centre, prospective study. J Hepatol 59:1054–1058. CrossRefPubMedGoogle Scholar
  2. 2.
    Chen Y, Nah SA, Chiang L, Krishnaswamy G, Low Y (2015) Postoperative steroid therapy for biliary atresia: systematic review and meta-analysis. J Pediatr Surg 50:1590–1594. CrossRefPubMedGoogle Scholar
  3. 3.
    Kobayashi H, Yamataka A, Koga H, Okazaki T, Tamura T, Urao M, Yanai T, Lane GJ, Miyano T (2005) Optimum prednisolone usage in patients with biliary atresia postportoenterostomy. J Pediatr Surg 40:327–330. CrossRefPubMedGoogle Scholar
  4. 4.
    Japanese Biliary Atresia S, Nio M, Muraji T (2013) Multicenter randomized trial of postoperative corticosteroid therapy for biliary atresia. Pediatr Surg Int 29:1091–1095. CrossRefGoogle Scholar
  5. 5.
    Murase N, Uchida H, Ono Y, Tainaka T, Yokota K, Tanano A, Shirota C, Shirotsuki R (2015) A new era of laparoscopic revision of Kasai portoenterostomy for the treatment of biliary atresia. Biomed Res Int 2015:173014. CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Karrer FM, Lilly JR (1985) Corticosteroid therapy in biliary atresia. J Pediatr Surg 20:693–695CrossRefPubMedGoogle Scholar
  7. 7.
    Bezerra JA, Spino C, Magee JC, Shneider BL, Rosenthal P, Wang KS, Erlichman J, Haber B, Hertel PM, Karpen SJ, Kerkar N, Loomes KM, Molleston JP, Murray KF, Romero R, Schwarz KB, Shepherd R, Suchy FJ, Turmelle YP, Whitington PF, Moore J, Sherker AH, Robuck PR, Sokol RJ, Childhood Liver Disease R, Education N (2014) Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA 311:1750–1759. CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Zhang MZ, Xun PC, He K, Cai W (2017) Adjuvant steroid treatment following Kasai portoenterostomy and clinical outcomes of biliary atresia patients: an updated meta-analysis. World J Pediatr 13:20–26. CrossRefPubMedGoogle Scholar
  9. 9.
    Miner PB Jr, Gaito JM (1979) Bile flow in response to pharmacologic agents. Hepatic DNA as a reference standard. Biochem Pharmacol 28:1063–1066CrossRefPubMedGoogle Scholar
  10. 10.
    Alvaro D, Gigliozzi A, Marucci L, Alpini G, Barbaro B, Monterubbianesi R, Minetola L, Mancino MG, Medina JF, Attili AF, Benedetti A (2002) Corticosteroids modulate the secretory processes of the rat intrahepatic biliary epithelium. Gastroenterology 122:1058–1069CrossRefPubMedGoogle Scholar
  11. 11.
    Dillon PW, Owings E, Cilley R, Field D, Curnow A, Georgeson K (2001) Immunosuppression as adjuvant therapy for biliary atresia. J Pediatr Surg 36:80–85. CrossRefPubMedGoogle Scholar
  12. 12.
    Suzuki T, Hashimoto T, Kondo S, Sato Y, Hussein MH (2010) Evaluating patients’ outcome post-Kasai operation: a 19-year experience with modification of the hepatic portoenterostomy and applying a novel steroid therapy regimen. Pediatr Surg Int 26:825–830. CrossRefPubMedGoogle Scholar
  13. 13.
    Yokota K, Uchida H, Tanano A, Shirota C, Tainaka T, Hinoki A, Murase N, Oshima K, Shirotsuki R, Chiba K (2016) Steroid pulse therapy prevents restenosis following balloon dilatation for esophageal stricture. Pediatr Surg Int 32:875–879. CrossRefPubMedGoogle Scholar
  14. 14.
    Narayanaswamy B, Gonde C, Tredger JM, Hussain M, Vergani D, Davenport M (2007) Serial circulating markers of inflammation in biliary atresia—evolution of the post-operative inflammatory process. Hepatology 46:180–187. CrossRefPubMedGoogle Scholar
  15. 15.
    Pang SY, Dai YM, Zhang RZ, Chen YH, Peng XF, Fu J, Chen ZR, Liu YF, Yang LY, Wen Z, Yu JK, Liu HY (2018) Autoimmune liver disease-related autoantibodies in patients with biliary atresia. World J Gastroenterol 24:387–396. CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Coburg AJ, Gray SH, Katz FH, Penn I, Halgrimson C, Starzl TE (1970) Disappearance rates and immunosuppression of intermittent intravenously administered prednisolone in rabbits and human beings. Surg Gynecol Obstet 131:933–942PubMedPubMedCentralGoogle Scholar
  17. 17.
    Shirota C, Uchida H, Ono Y, Murase N, Tainaka T, Yokota K, Oshima K, Shirotsuki R, Hinoki A, Ando H (2016) Long-term outcomes after revision of Kasai portoenterostomy for biliary atresia. J Hepatobiliary Pancreat Sci 23:715–720. CrossRefPubMedGoogle Scholar
  18. 18.
    Alonso EM, Ye W, Hawthorne K, Venkat V, Loomes KM, Mack CL, Hertel PM, Karpen SJ, Kerkar N, Molleston JP, Murray KF, Romero R, Rosenthal P, Schwarz KB, Shneider BL, Suchy FJ, Turmelle YP, Wang KS, Sherker AH, Sokol RJ, Bezerra JA, Magee JC, Chi LN (2018) Impact of steroid therapy on early growth in infants with biliary atresia: the multicenter steroids in biliary atresia randomized trial. J Pediatr 202(179–185):e174. CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Yujiro Tanaka
    • 1
    Email author
  • Chiyoe Shirota
    • 1
  • Takahisa Tainaka
    • 1
  • Wataru Sumida
    • 1
  • Kazuo Oshima
    • 1
  • Satoshi Makita
    • 1
  • Tomoko Tanaka
    • 1
  • Yukiko Tani
    • 1
  • Kosuke Chiba
    • 1
  • Hiroo Uchida
    • 1
  1. 1.Department of Pediatric SurgeryNagoya University Graduate School of MedicineNagoyaJapan

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