Pediatric Gastroenterology - Original Article

Revisiting long-term prognostic factors of biliary atresia: A 20-year experience with 81 patients from a single center

Volume 30 · Issue 5 Publish Date: May 15, 2019
Damla Hanalioğlu Hasan Özen Asuman Karhan Ersin Gümüş Hülya Demir İnci N. Saltık-Temizel Saniye Ekinci İbrahim Karnak Arbay O. Çiftçi Feridun C. Tanyel Aysel Yüce
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Turkish Journal of Gastroenterology

Volume 30 · Issue 5

Pages: 467-474

History

Published Online: May 15, 2019

Copyright © 2019 Damla Hanalioğlu, Hasan Özen, Asuman Karhan, Ersin Gümüş, Hülya Demir, İnci N. Saltık-Temizel, Saniye Ekinci, İbrahim Karnak, Arbay O. Çiftçi, Feridun C. Tanyel, Aysel Yüce

Damla Hanalioğlu
Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey
Hasan Özen
Departments of Pediatric Gastroenterology, Hepatology and Nutrition and Medical Microbiology, Hacettepe University School of Medicine, Ankara
Asuman Karhan
Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey
Ersin Gümüş
Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey
Hülya Demir
Department of Pediatrics, Section of Gastroenterology, Hacettepe University School of Medicine, Ankara
İnci N. Saltık-Temizel
Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey
Saniye Ekinci
Department of Pediatrics Surgery, Hacettepe University School of Medicine, Ankara, Turkey
İbrahim Karnak
Department of Pediatrics Surgery, Hacettepe University School of Medicine, Ankara, Turkey
Arbay O. Çiftçi
Department of Pediatrics Surgery, Hacettepe University School of Medicine, Ankara, Turkey
Feridun C. Tanyel
Department of Pediatrics Surgery, Hacettepe University School of Medicine, Ankara, Turkey
Aysel Yüce
Department of Pediatrics, Section of Gastroenterology, Hacettepe University School of Medicine, Ankara
Hanalioğlu, D., Özen, H., Karhan, A., Gümüş, E., Demir, H., Saltık-Temizel, İnci N., … Yüce, A. (2019). Revisiting long-term prognostic factors of biliary atresia: A 20-year experience with 81 patients from a single center. Turkish Journal of Gastroenterology, 30(5), 467–474. https://doi.org/10.5152/tjg.2019.18590
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Abstract

Abstract Background/Aims: The present study aimed at investigating the long-term outcomes and prognostic factors of patients with biliary atresia (BA) diagnosed and followed at a single center. Materials and Methods: Patients with BA treated during 1994-2014 at a large-volume pediatric tertiary referral center were reviewed retrospectively with regard to demographic, clinical, laboratory, and diagnostic characteristics for identifying the prognostic factors and long-term clinical outcomes. Results: Overall, 81 patients (49 males, 32 females) were included. Mean age at diagnosis was 73.1±4.7 (median: 64) days. Of the patients included, 78 patients (96%) underwent a portoenterostomy procedure. Mean age at operation was 76.8±4.7 (median: 72) days. The surgical success rate was 64.8%. A younger age (either at diagnosis or surgery) was the only determinant of surgical success. The 2-, 5-, and 10-year overall survival (OS) rates, including all patients with or without liver transplantation, were 75%, 73%, and 71% respectively, whereas the 2-, 5-, and 10-year survival rates with native liver (SNL) were 69%, 61%, and 57%, respectively. Mean follow-up duration was 9.4±7.5 years. Successful surgery, presence of fibrosis and/or cirrhosis on the liver pathology, and prothrombin time [international normalized ratio (INR)] at presentation were independent prognostic factors for both OS and SNL. Conclusion: A younger age at diagnosis is strongly associated with surgical success in BA. Surgical success, the prothrombin time (INR) at presentation, and liver pathology are independent prognostic factors affecting the long-term outcomes in patients with BA. Therefore, timely diagnosis and early referral to experienced surgical centers are crucial for optimal management and favorable long-term results in BA. Cite this article as: Hanalioğlu D, Özen H, Karhan A, et al. Revisiting long-term prognostic factors of biliary atresia: A 20-year experience with 81 patients from a single center. Turk J Gastroenterol 2019; 30(5): 467-74.

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Article Info
Published In
Journal Turkish Journal of Gastroenterology
Volume / Issue Volume 30 · Issue 5
Pages 467-474
DOI 10.5152/tjg.2019.18590
History
Published Online May 15, 2019
Copyright
Copyright (c) 2019 Damla Hanalioğlu, Hasan Özen, Asuman Karhan, Ersin Gümüş, Hülya Demir, İnci N. Saltık-Temizel, Saniye Ekinci, İbrahim Karnak, Arbay O. Çiftçi, Feridun C. Tanyel, Aysel Yüce
Affiliations
Damla Hanalioğlu
Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey
Hasan Özen
Departments of Pediatric Gastroenterology, Hepatology and Nutrition and Medical Microbiology, Hacettepe University School of Medicine, Ankara
Asuman Karhan
Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey
Ersin Gümüş
Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey
Hülya Demir
Department of Pediatrics, Section of Gastroenterology, Hacettepe University School of Medicine, Ankara
İnci N. Saltık-Temizel
Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Hacettepe University School of Medicine, Ankara, Turkey
Saniye Ekinci
Department of Pediatrics Surgery, Hacettepe University School of Medicine, Ankara, Turkey
İbrahim Karnak
Department of Pediatrics Surgery, Hacettepe University School of Medicine, Ankara, Turkey
Arbay O. Çiftçi
Department of Pediatrics Surgery, Hacettepe University School of Medicine, Ankara, Turkey
Feridun C. Tanyel
Department of Pediatrics Surgery, Hacettepe University School of Medicine, Ankara, Turkey
Aysel Yüce
Department of Pediatrics, Section of Gastroenterology, Hacettepe University School of Medicine, Ankara
Cite this Article
Hanalioğlu, D., Özen, H., Karhan, A., Gümüş, E., Demir, H., Saltık-Temizel, İnci N., … Yüce, A. (2019). Revisiting long-term prognostic factors of biliary atresia: A 20-year experience with 81 patients from a single center. Turkish Journal of Gastroenterology, 30(5), 467–474. https://doi.org/10.5152/tjg.2019.18590
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