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Biliary atresia. [PDF]

Nat Rev Dis Primers
Biliary atresia (BA) is a progressive inflammatory fibrosclerosing disease of the biliary system and a major cause of neonatal cholestasis. It affects 1:5,000-20,000 live births, with the highest incidence in Asia. The pathogenesis is still unknown, but emerging research suggests a role for ciliary dysfunction, redox stress and hypoxia.
Tam PKH, Wells RG, Tang CSM, Lui VCH, Hukkinen M, Luque CD, De Coppi P, Mack CL, Pakarinen M, Davenport M.   +9 more
europepmc   +4 more sources

Current and emerging adjuvant therapies in biliary atresia

Frontiers in Pediatrics, 2022
Following Kasai hepatic portoenterostomy (HPE), most patients with biliary atresia will eventually require liver transplantation due to progressive cirrhosis and liver failure.
Scott C. Fligor, Thomas I. Hirsch, Savas T. Tsikis, Andrew Adeola, Mark Puder   +4 more
doaj   +2 more sources

Ultrasound for the Diagnosis of Biliary Atresia: From Conventional Ultrasound to Artificial Intelligence

Diagnostics, 2021
Biliary atresia is an aggressive liver disease of infancy and can cause death without timely surgical intervention. Early diagnosis of biliary atresia is critical to the recovery of bile drainage and long-term transplant-free survival.
Wenying Zhou, Luyao Zhou
doaj   +2 more sources

Prognosis of Biliary Atresia Associated With Cytomegalovirus: A Meta-Analysis

Frontiers in Pediatrics, 2021
Objective: The etiology of biliary atresia is unclear, but viral infection has been implicated. The aim of the current meta-analysis was to investigate relationships between cytomegalovirus (CMV) and the prognosis of biliary atresia.Methods: PubMed ...
Yilin Zhao, Yilin Zhao, Xiaodan Xu, Xiaodan Xu, Gengxin Liu, Gengxin Liu, Fang Yang, Fang Yang, Jianghua Zhan   +8 more
doaj   +2 more sources

Impacts of Early Kasai Portoenterostomy on Short‐Term and Long‐Term Outcomes of Biliary Atresia

Hepatology Communications, 2021
There are discrepancies regarding the clinical impact of age at Kasai portoenterostomy (KP) on surgical outcomes. Hence, we re‐assessed the clinical significance of age at KP.
Ryuji Okubo, Masaki Nio, Hideyuki Sasaki, the Japanese Biliary Atresia Society   +3 more
doaj   +2 more sources

Biliary atresia: pathology, etiology and pathogenesis

Future Science OA, 2020
Biliary atresia is a progressive fibrosing obstructive cholangiopathy of the intrahepatic and extrahepatic biliary system, resulting in obstruction of bile flow and neonatal jaundice.
Mukul Vij, Mohamed Rela
doaj   +2 more sources

Association of Gut Microbiota and Metabolites With Disease Progression in Children With Biliary Atresia

Frontiers in Immunology, 2021
Background and AimsBiliary atresia is the most common cause of liver disease and liver transplantation in children. The accumulation of bile acids in hepatocytes and the stimulation of the intestinal microbiome can aggravate the disease progression. This
Wei Song, Wei Song, Li-Ying Sun, Li-Ying Sun, Li-Ying Sun, Zhi-Jun Zhu, Zhi-Jun Zhu, Lin Wei, Lin Wei, Wei Qu, Wei Qu, Zhi-Gui Zeng, Zhi-Gui Zeng, Ying Liu, Ying Liu, Hai-Ming Zhang, Hai-Ming Zhang, Wei Guo   +17 more
doaj   +2 more sources

Japanese biliary atresia registry [PDF]

World Journal of Pediatric Surgery
The Japanese Biliary Atresia Registry (JBAR) was launched in 1989 by the Japanese Biliary Atresia Society (JBAS) to investigate the epidemiology and etiology of biliary atresia and to improve surgical outcomes.
Masaki Nio, Ryuji Okubo, Hideyuki Sasaki, Motoshi Wada   +3 more
doaj   +2 more sources

Development and validation of bile acid profile-based scoring system for identification of biliary atresia: a prospective study

BMC Pediatrics, 2020
Background Early distinguishing biliary atresia from other causes of infantile cholestasis remains a major challenge. We aimed to develop and validate a scoring system based on bile acid for identification of biliary atresia.
Dongying Zhao, Kejun Zhou, Yan Chen, Wei Xie, Yongjun Zhang   +4 more
doaj   +2 more sources

Outcomes of Liver Transplantation in Patients With Congenital Heart Disease and Biliary Atresia. A Multicenter Analysis. [PDF]

Pediatr Transplant
Liver transplantation in patients with CHD and BA is safe. Although patients with CHD display heightened utilization of in‐hospital resources, no discernible variance in long‐term outcomes was observed. ABSTRACT Introduction Congenital heart disease (CHD) frequently coexists with noncardiac malformations.
O'Connor M, Adler JT, Venardos NM, Ponce-Rivera MS, Fraser CD, Mery CM, Well A.   +6 more
europepmc   +2 more sources