Liver transplantation for deterioration in native liver function after portoenterostomy for biliary atresia in Japan: Short- versus long-term survivors.

Abstract:

PURPOSE:We reviewed our post-Kasai portoenterostomy biliary atresia (BA) patients who required liver transplantation (LTx) for deterioration in native liver (NL) function to investigate mortality in relation to age at LTx. METHODS:BA patients indicated for LTx when less than 18years old (U18; n=17) and when 18 or older (18+; n=13) were compared. All achieved jaundice clearance postoperatively (TBil ≤1.2mg/dL (≈20μmol/L)). RESULTS:In U18, living-donor (LD) LTxs were performed at a median of 6.1years (range: 0.5-16.7; n=14) and cadaveric (CD) LTxs at a median of 1.3years (1.1-1.5; n=3). In 18+, LDLTxs were performed at a median of 28years (18-37; n=8), and 1 case died from graft versus host disease. CDLTxs were indicated in 5, but 4 died at a median of 30years (26-32), a mean of 1.4years (0.7-1.8) after NL deterioration commenced. One case is awaiting CDLTx. At the time of review, all U18 and 7 LDLTx cases in 18+ were clinically stable. Mortality rates were 0% in U18 and 38% in 18+ (P=.006). CONCLUSION:Our results highlight the extremely grave prognosis for long-term BA patients requiring LTx when 18 or older because of poor donor availability in Japan. LEVEL OF EVIDENCE:Level III.

journal_name

J Pediatr Surg

authors

Ochi T,Nakamura H,Wada M,Tamura T,Koga H,Okazaki T,Urao M,Ishizaki Y,Kawasaki S,Kasahara M,Mizuta K,Lane GJ,Yamataka A

doi

10.1016/j.jpedsurg.2017.11.016

subject

Has Abstract

pub_date

2018-02-01 00:00:00

pages

277-280

issue

2

eissn

0022-3468

issn

1531-5037

pii

S0022-3468(17)30734-0

journal_volume

53

pub_type

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