Accumulation of altered serum bile acids predicts liver injury after portoenterostomy in biliary atresia
Biliary atresia, a fibro-obliterating biliary disease of infants, remains the most common indication for pediatric liver transplantation because of the rapid progression of liver injury. To identify predictive biomarkers of disease progression and to elucidate the pathophysiology of liver injury in biliary atresia, we profiled serum bile acids and studied their liver metabolism after Kasai portoenterostomy. Accumulation and altered composition of circulating bile acids predicted progression of liver disease and need for liver transplantation. Patients with poor prognosis showed low bile acid synthesis and abnormal liver expression of fibroblast growth factor 19.
Authors
- Iiris Nyholm (ORCID: https://orcid.org/0000-0003-4999-4280)
- Satu M. Hänninen (ORCID: https://orcid.org/0000-0002-2758-8444)
- Mikko P. Pakarinen (ORCID: https://orcid.org/0000-0001-6016-6334)
- Olli Carpén (ORCID: https://orcid.org/0000-0002-7847-5706)
- Timo Jahnukainen (ORCID: https://orcid.org/0000-0002-1815-7327)
- Nelli Sjöblom (ORCID: https://orcid.org/0000-0002-3360-6645)
- Mikko Niemi (ORCID: https://orcid.org/0000-0003-0461-3667)
- Markku Heikinheimo (ORCID: https://orcid.org/0000-0001-9452-0661)
- Aino Mutka (ORCID: https://orcid.org/0000-0002-2885-3211)
- Maria Hukkinen (ORCID: https://orcid.org/0000-0002-2622-8391)
- Satu Ruuska (ORCID: https://orcid.org/0000-0002-2768-855X)
- Johanna Arola (ORCID: https://orcid.org/0000-0003-3865-5507)
- Mikko Neuvonen (ORCID: https://orcid.org/0000-0002-7627-1638)
- Annika Mutanen (ORCID: https://orcid.org/0000-0003-1575-5601)
- Jouko Lohi
Institutions
- University of Helsinki (FI)
- Helsinki University Hospital (FI)
- Helsinki Children's Hospital (FI)
- Tampere University Hospital (FI)
Publication Details
- Journal
- Journal of Hepatology
- Published
- 2025-01-29
- DOI
- https://doi.org/10.1016/j.jhep.2025.01.025
- Citations
- 10
- Primary Topic
- Pediatric Hepatobiliary Diseases and Treatments
- Type
- article
- Field-Weighted Citation Impact
- 20.06
Funders
- Helsingin ja Uudenmaan Sairaanhoitopiiri
- Mary och Georg C. Ehrnrooths Stiftelse
- Lastentautien Tutkimussäätiö
- Sigrid Juséliuksen Säätiö