Observational Study
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Nov 21, 2017; 23(43): 7776-7784
Published online Nov 21, 2017. doi: 10.3748/wjg.v23.i43.7776
Chronic liver disease is universal in children with biliary atresia living with native liver
Way Seah Lee, Sik Yong Ong, Hee Wei Foo, Shin Yee Wong, Chen Xi Kong, Ru Bin Seah, Ruey Terng Ng
Way Seah Lee, Sik Yong Ong, Hee Wei Foo, Shin Yee Wong, Chen Xi Kong, Ru Bin Seah, Ruey Terng Ng, Department of Paediatrics, University Malaya Medical Centre, Kuala Lumpur 59100, Malaysia
Way Seah Lee, Paediatrics and Child Health Research Group, University Malaya, Kuala Lumpur 50603, Malaysia
Author contributions: Lee WS and Ong SY conceived the concept of the present research; Ong SY, Foo HW, Kong CX, Seah RB and Ng RT collected the clinical data; Wong SY provided statistical analysis; Lee WS and Ong SY wrote the first draft; Lee WS provided critical analysis to the draft; Lee WS revised the final draft; All authors approved the final draft.
Supported by University Malaya High Impact Research (UM.C/625/HIR/MOHE/CHAN/13/1) from Ministry of Higher Education, Malaysia.
Institutional review board statement: The study was reviewed and approved by the University Malaya Medical Centre, Kuala Lumpur Medical Ethical Committee (MEC 902.15).
Informed consent statement: All study participants, or their legal guardian, provided informed written consent prior to study enrolment.
Conflict-of-interest statement: None of the authors has any potential conflict of interest to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Way Seah Lee, MBBS, MD, FRCPCH, Professor, Department of Paediatrics, University Malaya Medical Centre, Kuala Lumpur 50603, Malaysia. leews@um.edu.my
Telephone: +603-79492065 Fax: +603-79494704
Received: June 9, 2017
Peer-review started: June 10, 2017
First decision: July 13, 2017
Revised: August 3, 2017
Accepted: August 15, 2017
Article in press: August 15, 2017
Published online: November 21, 2017
Processing time: 164 Days and 18 Hours
Abstract
AIM

To examine the medical status of children with biliary atresia (BA) surviving with native livers.

METHODS

In this cross-sectional review, data collected included complications of chronic liver disease (CLD) (cholangitis in the preceding 12 mo, portal hypertension, variceal bleeding, fractures, hepatopulmonary syndrome, portopulmonary hypertension) and laboratory indices (white cell and platelet counts, total bilirubin, albumin, international normalized ratio, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase). Ideal medical outcome was defined as absence of clinical evidence of CLD or abnormal laboratory indices.

RESULTS

Fifty-two children [females = 32, 62%; median age 7.4 years, n = 35 (67%) older than 5 years] with BA (median age at surgery 60 d, range of 30 to 148 d) survived with native liver. Common complications of CLD noted were portal hypertension (40%, n = 21; 2 younger than 5 years), cholangitis (36%) and bleeding varices (25%, n = 13; 1 younger than 5 years). Fifteen (29%) had no clinical complications of CLD and three (6%) had normal laboratory indices. Ideal medical outcome was only seen in 1 patient (2%).

CONCLUSION

Clinical or laboratory evidence of CLD are present in 98% of children with BA living with native livers after hepatoportoenterostomy. Portal hypertension and variceal bleeding may be seen in children younger than 5 years of age, underscoring the importance of medical surveillance for complications of BA starting at a young age.

Keywords: Biliary atresia; Medical status; Chronic liver disease

Core tip: Previous study showed that more than 90% of children with biliary atresia (BA) surviving with native livers have clinical and laboratory evidence of chronic liver disease (CLD). In the present cohort, we found that 71% of patients with BA living with native livers had no clinical complications of CLD and 90% had normal liver synthetic function, only 2% had ideal medical outcome. Common medical complications encountered were cholangitis, portal hypertension and bleeding oesophageal varices. Portal hypertension and bleeding oesophageal varices were seen in 12% and 6% of children younger than 5 years of age. Medical surveillance in children with BA after Kasai surgery for medical complications should start even before 5 years of age.