Abstract
Background
We reviewed the time taken for post-portoenterostomy (PE) biliary atresia (BA) patients to obtain jaundice-clearance (total bilirubin ≤ 1.2 mg/dL; JC) post-PE to determine if JC time (JCT) is prognostic for survival of the native liver (SNL).
Methods
The subjects were 66 BA patients treated with PE at our institute between 1989, the year when liver transplantation (LTx) became available in Japan, and 2014. JCT was used to create three groups (≤ 30 days: n = 14; 31–60 days: n = 31; ≥ 61 days: n = 21). Medical records were reviewed retrospectively to evaluate: age at onset of symptoms, duration of symptoms pre-PE, age and weight at PE, serum liver function tests, incidence of cholangitis, and micro-bile duct size at PE.
Results
Age at onset of symptoms, age and weight at PE, duration of symptoms pre-PE, and micro-bile duct size were similar for all patients in all three groups. JCT and SNL appeared to correlate because preoperative total bilirubin (7.1, 9.6, 10.2 mg/dL; P < 0.05) was significantly lower in the JCT ≤ 30 days group (P < 0.05) while there was a significant decrease in SNL (P < 0.03) and a significant increase in LTx (P < 0.01) in the JCT ≥ 61 days group. All LTx subjects who achieved JC were found to have developed cholangitis within 3 months of PE.
Conclusion
During the follow-up of post-PE subjects, longer JCT and cholangitis occurrence within 3 months of PE would appear to be negative prognostic factors for SNL while preoperative total bilirubin would appear to be a positive prognostic factor for SNL.
Similar content being viewed by others
References
Hartley JL, Davenport M, Kelly DA. Biliary atresia. Lancet. 2009;374:1704–13.
Howard ER, MacLean G, Nio M, Donaldson N, Singer J, Ohi R. Survival patterns in biliary atresia and comparison of quality of life of long-term survivors in Japan and England. J Pediatr Surg. 2001;36:892–7.
Lykavieris P, Chardot C, Sokhn M, Gauthier F, Valayer J, Bernard O. Outcome in adulthood of biliary atresia: a study of 63 patients who survived for over 20 years with their native liver. Hepatology. 2005;41:366–71.
Koga H, Wada M, Nakamura H, Miyano G, Okawada M, Lane GJ, et al. Factors influencing jaundice-free survival with the native liver in post-portoenterostomy biliary atresia patients: results from a single institution. J Pediatr Surg. 2013;48:2368–72.
Nakamura H, Koga H, Wada M, Miyano G, Dizon R, Kato Y, et al. Reappraising the portoenterostomy procedure according to sound physiologic/anatomic principles enhances postoperative jaundice clearance in biliary atresia. Pediatr Surg Int. 2012;28:205–9.
Ohhama Y, Shinkai M, Fujita S, Nishi T, Yamamoto H. Early prediction of long-term survival and the timing of liver transplantation after the Kasai operation. J Pediatr Surg. 2000;35:1031–4.
Superina R, Magee JC, Brandt ML, Healey PJ, Tiao G, Ryckman F, et al. The anatomic pattern of biliary atresia identified at time of Kasai hepatoportoenterostomy and early postoperative clearance of jaundice are significant predictors of transplant-free survival. Ann Surg. 2011;254:577–85.
Oh M, Hobeldin M, Chen T, Thomas DW. Atkinson JB. The Kasai procedure in the treatment of biliary atresia. J Pediatr Surg. 1995;30:1077–80.
Nio M, Wada M, Sasaki H, Tanaka H, Okamura A. Risk factors affecting late-presenting liver failure in adult patients with biliary atresia. J Pediatr Surg. 2012;47:2179–83.
Rodeck B, Becker AC, Gratz KF, Petersen C. Early predictors of success of Kasai operation in children with biliary atresia. Eur J Pediatr Surg. 2007;7:308–12.
Shneider BL, Brown MB, Haber B, Whitington PF, Scwarz K, Squirres R, et al. A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000. J Pediatr. 2006;148:467–74.
Vazquez-Estevez J, Stewart B, Shikes RH, Hall RJ, Lilly JR. Biliary atresia: early determination of prognosis. J Pediatr Surg. 1989;24:48–50; discussion 50–1.
Goda T, Kawahara H, Kubota A, Hirano K, Umeda S, Tani S, et al. The most reliable early predictors of outcome in patients with biliary atresia after Kasai’s operation. J Pediatr Surg. 2013;48:2373–7.
Iwatsuki S, Shaw BW Jr, Starzl TE. Liver transplantation for biliary atresia. World J Surg. 1984;8:51–6.
Kubota A, Okada A, Fukui Y, Kawahara H, Imura K. Kamata S. Indocyanine green test is a reliable indicator of postoperative liver function in biliary atresia. J Pediatr Gastroenterol Nutr. 1993;16:61–5.
Altman RP, Lilly JR, Greenfeld J, Weinberg A, van Leeuwen K, Flanigan L. A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: twenty-five years of experience from two centers. Ann Surg. 1997;226:348–53.
Azarow KS, Phillips MJ, Sandler AD, Hagerstrand I, Superina RA. Biliary atresia: should all patients undergo a portoenterostomy? J Pediatr Surg. 1997;32:168–72.
Nio M, Ohi R, Miyano T, Saeki T, Shiraki K, Tanaka K, et al. Five- and 10-year survival rates after surgery for biliary atresia: a report from the Japanese Biliary Atresia Registry. J Pediatr Surg. 2003;38:997–1000.
Shinkai M, Ohhama Y, Take H, Kitagawa N, Kudo H, Mochizuki K, et al. Long-term outcome of children with biliary atresia who were not transplanted after the Kasai operation: > 20-year experience at a children’s hospital. J Pediatr Gastroenterol Nutr. 2009;48:443–50.
Hung PY, Chen CC, Chen WJ, Lai HS, Hsu WM, Lee PH, et al. Long-term prognosis of patients with biliary atresia: a 25 year summary. J Pediatr Gastroenterol Nutr. 2006;42:190–5.
Bu LN, Chen HL, Ni YH, Peng S, Jeng YM, Lai HS, et al. Multiple intrahepatic biliary cysts in children with biliary atresia. J Pediatr Surg. 2002;37:1183–7.
Wu ET, Chen HL, Ni YH, Lee PI, Hsu HY, Lai HS, et al. Bacterial cholangitis in patients with biliary atresia: impact on short-term outcome. Pediatr Surg Int. 2001;17:390–5.
Funding
No funding was acquired or utilized for this study.
Author information
Authors and Affiliations
Contributions
HN contributed to the collection of data, and wrote the first version of the paper. HK contributed to the design, data analysis of this study, and wrote the first version of the paper. MO and HN contributed to the collection of data. GJL wrote the first version of the paper. AY contributed to the design and data analysis of this study. All authors approved the final version of the manuscript.
Corresponding author
Ethics declarations
Ethical approval
This study was approved by the Juntendo University School of Medicine Institutional Review Board and complies with the Helsinki Declaration of 1975 (revised 1983).
Conflict of interest
The authors declare no conflict of interest.
Rights and permissions
About this article
Cite this article
Nakajima, H., Koga, H., Okawada, M. et al. Does time taken to achieve jaundice-clearance influence survival of the native liver in post-Kasai biliary atresia?. World J Pediatr 14, 191–196 (2018). https://doi.org/10.1007/s12519-018-0139-5
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s12519-018-0139-5