Abstract
BackgroundBiliary atresia (BA) is a typical cholestatic neonatal disease, characterized by obliteration of intra- and/or extra-hepatic bile ducts. However, the mechanisms contributing to the pathogenesis of BA remain uncertain. Because of decreased bile flow, infectious complications and damaging endotoxemia occur frequently in patients with BA. The aim of this study was to investigate endotoxin levels in patients with BA and the relation of these levels with the expression of the endotoxin receptor, CD14.MethodsThe plasma levels of endotoxin and soluble CD14 were measured with a pyrochrome Limulus amebocyte lysate assay and enzyme-linked immunosorbent assay in patients with early-stage BA when they received the Kasai procedure (KP), in patients who were jaundice-free post-KP and followed-up at the outpatient department, in patients with late-stage BA when they received liver transplantation, and in patients with choledochal cysts. The correlation of CD14 expression with endotoxin levels in rats following common bile duct ligation was investigated.ResultsThe results demonstrated a significantly higher hepatic CD14 mRNA and soluble CD14 plasma levels in patients with early-stage BA relative to those with late-stage BA. However, plasma endotoxin levels were significantly higher in both the early and late stages of BA relative to controls. In rat model, the results demonstrated that both endotoxin and CD14 levels were significantly increased in liver tissues of rats following bile duct ligation.ConclusionsThe significant increase in plasma endotoxin and soluble CD14 levels during BA implies a possible involvement of endotoxin stimulated CD14 production by hepatocytes in the early stage of BA for removal of endotoxin; whereas, endotoxin signaling likely induced liver injury and impaired soluble CD14 synthesis in the late stages of BA.
Highlights
Biliary atresia (BA) is a typical cholestatic neonatal disease, characterized by obliteration of intra- and/ or extra-hepatic bile ducts
Plasma CD14 and endotoxin levels in patients with BA Plasma soluble CD14 (sCD14) levels were analyzed by enzyme-linked immunosorbent assay (ELISA) and found to be significantly higher in patients with early-stage BA
SCD14 levels in the early-stage BA, outpatient department (OPD), and choledochal cysts (CCs) groups were higher than controls (3879 ± 767 ng/ml), these differences were not statistically significant (P = 0.134, P = 0.447, and P = 0.442, respectively) (Figure 1)
Summary
Biliary atresia (BA) is a typical cholestatic neonatal disease, characterized by obliteration of intra- and/ or extra-hepatic bile ducts. Biliary atresia (BA) is a typical cholestatic neonatal disease, characterized by obliteration of intra- and/or extra-hepatic bile ducts with repeated episodes of cholangitis and progressive liver fibrosis and cirrhosis [1,2,3]. LPS represent the major component of the outer membrane of Gramnegative bacteria and has been implicated in sepsis, CD14 is a glycosylphosphatidylinositol-anchored LPS receptor. It was first reported as a differentiation marker expressed on the surface of macrophages, neutrophils, and other myeloid lineage cells [10,11,12,13]. The proportional change of CD14 production in the liver and the subsequent effects on LPS-induced liver injury during BA is not clear
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
