Serum bile acids as a prognostic biomarker in biliary atresia following Kasai portoenterostomy

for Childhood Liver Disease Research Network (ChiLDReN)

doi:10.1002/hep.32800

Serum bile acids as a prognostic biomarker in biliary atresia following Kasai portoenterostomy

for Childhood Liver Disease Research Network (ChiLDReN)

Pediatrics

Research output: Contribution to journal › Article › peer-review

13 Scopus citations

Abstract

Background and Aims: In biliary atresia, serum bilirubin is commonly used to predict outcomes after Kasai portoenterostomy (KP). Infants with persistently high levels invariably need liver transplant, but those achieving normalized levels have a less certain disease course. We hypothesized that serum bile acid levels could help predict outcomes in the latter group. Approach and Results: Participants with biliary atresia from the Childhood Liver Disease Research Network were included if they had normalized bilirubin levels 6 months after KP and stored serum samples from the 6-month post-KP clinic visit (n = 137). Bile acids were measured from the stored serum samples and used to divide participants into ≤40 μmol/L (n = 43) or >40 μmol/L (n = 94) groups. At 2 years of age, the ≤40 μmol/L compared with >40 μmol/L group had significantly lower total bilirubin, aspartate aminotransferase, alanine aminotransferase, gamma-glutamyltransferase, bile acids, and spleen size, as well as significantly higher albumin and platelet counts. Furthermore, during 734 person-years of follow-up, those in the ≤40 μmol/L group were significantly less likely to develop splenomegaly, ascites, gastrointestinal bleeding, or clinically evident portal hypertension. The ≤40 μmol/L group had a 10-year cumulative incidence of liver transplant/death of 8.5% (95% CI: 1.1%-26.1%), compared with 42.9% (95% CI: 28.6%-56.4%) for the >40 μmol/L group (p = 0.001). Conclusions: Serum bile acid levels may be a useful prognostic biomarker for infants achieving normalized bilirubin levels after KP.

Original language	English (US)
Pages (from-to)	862-873
Number of pages	12
Journal	Hepatology
Volume	77
Issue number	3
DOIs	https://doi.org/10.1002/hep.32800
State	Published - Mar 2023

Funding

Supported by the National Institute of Diabetes, Digestive, and Kidney Diseases (U01 grants DK103149 to Baylor College of Medicine, DK062470 to Children's Healthcare of Atlanta, DK103135 to The Hospital for Sick Children, DK062436 to Ann & Robert H. Lurie Children's Hospital of Chicago, DK062497 to Cincinnati Children's Hospital Medical Center, DK103140 to University of Utah, DK084575 to Seattle Children's Hospital, DK062481 to the Children's Hospital of Philadelphia, DK062466 to Children's Hospital of Pittsburgh of the University of Pittsburgh Medical Center, DK062456 to University of Michigan, DK084536 to Riley Hospital for Children, DK062500 to University of California San Francisco Children's Hospital, DK084538 to Children's Hospital Los Angeles, DK062453 to Children's Hospital Colorado) and the National Institutes of Health, National Center for Advancing Translational Sciences, Clinical and Translational Sciences Awards (UL1TR002378 to Children's Healthcare of Atlanta, UL1 TR000423 and UL1 RR025014 to Seattle Children's Hospital, UL1 TR001857 to Children's Hospital of Pittsburgh of UPMC, UL1 TR001878 and TR000003 to the Children's Hospital of Philadelphia, UL1 TR001872 to UCSF Children's Hospital, UL1TR00130 to Children's Hospital of Los Angeles, and UL1 TR002535 to University of Colorado). SH is also funded by NIH K23DK109207 and R03DK128535 as well as generous philanthropic support from Robert and Annie Graham.

ASJC Scopus subject areas

Hepatology

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10.1002/hep.32800

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@article{6c26572d003f49ac8ee8dd4bdff0b1b1,

title = "Serum bile acids as a prognostic biomarker in biliary atresia following Kasai portoenterostomy",

abstract = "Background and Aims: In biliary atresia, serum bilirubin is commonly used to predict outcomes after Kasai portoenterostomy (KP). Infants with persistently high levels invariably need liver transplant, but those achieving normalized levels have a less certain disease course. We hypothesized that serum bile acid levels could help predict outcomes in the latter group. Approach and Results: Participants with biliary atresia from the Childhood Liver Disease Research Network were included if they had normalized bilirubin levels 6 months after KP and stored serum samples from the 6-month post-KP clinic visit (n = 137). Bile acids were measured from the stored serum samples and used to divide participants into ≤40 μmol/L (n = 43) or >40 μmol/L (n = 94) groups. At 2 years of age, the ≤40 μmol/L compared with >40 μmol/L group had significantly lower total bilirubin, aspartate aminotransferase, alanine aminotransferase, gamma-glutamyltransferase, bile acids, and spleen size, as well as significantly higher albumin and platelet counts. Furthermore, during 734 person-years of follow-up, those in the ≤40 μmol/L group were significantly less likely to develop splenomegaly, ascites, gastrointestinal bleeding, or clinically evident portal hypertension. The ≤40 μmol/L group had a 10-year cumulative incidence of liver transplant/death of 8.5% (95% CI: 1.1%-26.1%), compared with 42.9% (95% CI: 28.6%-56.4%) for the >40 μmol/L group (p = 0.001). Conclusions: Serum bile acid levels may be a useful prognostic biomarker for infants achieving normalized bilirubin levels after KP.",

author = "{for Childhood Liver Disease Research Network (ChiLDReN)} and Sanjiv Harpavat and Kieran Hawthorne and Setchell, {Kenneth D.R.} and Monica Narvaez Rivas and Lisa Henn and Beil, {Charlotte A.} and Karpen, {Saul J.} and Vicky L Ng and Alonso, {Estella M.} and Bezerra, {Jorge A.} and Guthery, {Stephen L.} and Simon Horslen and Loomes, {Kathy M.} and Patrick McKiernan and John C Magee and Merion, {Robert M.} and Molleston, {Jean P.} and Philip Rosenthal and Thompson, {Richard J.} and Wang, {Kasper S.} and Sokol, {Ronald J.} and Benjamin L Shneider",

year = "2023",

month = mar,

doi = "10.1002/hep.32800",

language = "English (US)",

volume = "77",

pages = "862--873",

journal = "Hepatology",

issn = "0270-9139",

publisher = "Lippincott Williams and Wilkins",

number = "3",

}

TY - JOUR

T1 - Serum bile acids as a prognostic biomarker in biliary atresia following Kasai portoenterostomy

AU - for Childhood Liver Disease Research Network (ChiLDReN)

AU - Harpavat, Sanjiv

AU - Hawthorne, Kieran

AU - Setchell, Kenneth D.R.

AU - Rivas, Monica Narvaez

AU - Henn, Lisa

AU - Beil, Charlotte A.

AU - Karpen, Saul J.

AU - Ng, Vicky L

AU - Alonso, Estella M.

AU - Bezerra, Jorge A.

AU - Guthery, Stephen L.

AU - Horslen, Simon

AU - Loomes, Kathy M.

AU - McKiernan, Patrick

AU - Magee, John C

AU - Merion, Robert M.

AU - Molleston, Jean P.

AU - Rosenthal, Philip

AU - Thompson, Richard J.

AU - Wang, Kasper S.

AU - Sokol, Ronald J.

AU - Shneider, Benjamin L

PY - 2023/3

Y1 - 2023/3

N2 - Background and Aims: In biliary atresia, serum bilirubin is commonly used to predict outcomes after Kasai portoenterostomy (KP). Infants with persistently high levels invariably need liver transplant, but those achieving normalized levels have a less certain disease course. We hypothesized that serum bile acid levels could help predict outcomes in the latter group. Approach and Results: Participants with biliary atresia from the Childhood Liver Disease Research Network were included if they had normalized bilirubin levels 6 months after KP and stored serum samples from the 6-month post-KP clinic visit (n = 137). Bile acids were measured from the stored serum samples and used to divide participants into ≤40 μmol/L (n = 43) or >40 μmol/L (n = 94) groups. At 2 years of age, the ≤40 μmol/L compared with >40 μmol/L group had significantly lower total bilirubin, aspartate aminotransferase, alanine aminotransferase, gamma-glutamyltransferase, bile acids, and spleen size, as well as significantly higher albumin and platelet counts. Furthermore, during 734 person-years of follow-up, those in the ≤40 μmol/L group were significantly less likely to develop splenomegaly, ascites, gastrointestinal bleeding, or clinically evident portal hypertension. The ≤40 μmol/L group had a 10-year cumulative incidence of liver transplant/death of 8.5% (95% CI: 1.1%-26.1%), compared with 42.9% (95% CI: 28.6%-56.4%) for the >40 μmol/L group (p = 0.001). Conclusions: Serum bile acid levels may be a useful prognostic biomarker for infants achieving normalized bilirubin levels after KP.

AB - Background and Aims: In biliary atresia, serum bilirubin is commonly used to predict outcomes after Kasai portoenterostomy (KP). Infants with persistently high levels invariably need liver transplant, but those achieving normalized levels have a less certain disease course. We hypothesized that serum bile acid levels could help predict outcomes in the latter group. Approach and Results: Participants with biliary atresia from the Childhood Liver Disease Research Network were included if they had normalized bilirubin levels 6 months after KP and stored serum samples from the 6-month post-KP clinic visit (n = 137). Bile acids were measured from the stored serum samples and used to divide participants into ≤40 μmol/L (n = 43) or >40 μmol/L (n = 94) groups. At 2 years of age, the ≤40 μmol/L compared with >40 μmol/L group had significantly lower total bilirubin, aspartate aminotransferase, alanine aminotransferase, gamma-glutamyltransferase, bile acids, and spleen size, as well as significantly higher albumin and platelet counts. Furthermore, during 734 person-years of follow-up, those in the ≤40 μmol/L group were significantly less likely to develop splenomegaly, ascites, gastrointestinal bleeding, or clinically evident portal hypertension. The ≤40 μmol/L group had a 10-year cumulative incidence of liver transplant/death of 8.5% (95% CI: 1.1%-26.1%), compared with 42.9% (95% CI: 28.6%-56.4%) for the >40 μmol/L group (p = 0.001). Conclusions: Serum bile acid levels may be a useful prognostic biomarker for infants achieving normalized bilirubin levels after KP.

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U2 - 10.1002/hep.32800

DO - 10.1002/hep.32800

M3 - Article

C2 - 36131538

AN - SCOPUS:85139618941

SN - 0270-9139

VL - 77

SP - 862

EP - 873

JO - Hepatology

JF - Hepatology

IS - 3

ER -

Serum bile acids as a prognostic biomarker in biliary atresia following Kasai portoenterostomy

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