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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Cell+Mol+Gastroenterol+Hepatol
2015 ; 1
(3
): 267-274
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What Causes Biliary Atresia? Unique Aspects of the Neonatal Immune System Provide
Clues to Disease Pathogenesis
#MMPMID26090510
Mack CL
Cell Mol Gastroenterol Hepatol
2015[May]; 1
(3
): 267-274
PMID26090510
show ga
Biliary atresia (BA) is the most frequent identifiable cause of neonatal
cholestasis and the majority of patients will need liver transplantation for
survival. Despite surgical intervention with the Kasai portoenterostomy,
significant fibrosis and cirrhosis develops early in life. An increased
understanding of what causes this inflammatory fibrosing cholangiopathy will lead
to therapies aimed at protecting the intrahepatic biliary system from
immune-mediated damage. This review focuses on studies pertaining to the role of
the adaptive immune response in bile duct injury in BA, including cellular and
humoral immunity. The neonatal presentation of BA begs the question of what are
potential modifications of unique aspects of the neonatal immune system that
"sets the stage" for the progressive biliary disease? Throughout this article,
characteristics of the neonatal immune response are outlined and theories as to
how alterations of this response could contribute to the pathogenesis of BA are
discussed. These include aberrant Th1 and Th17 responses, deficiencies in
regulatory T cells, activation of humoral immunity and autoimmunity. In order to
advance our understanding of the etiology of BA, future studies should focus on
those unique aspects of the neonatal immune system that have gone awry, as
detailed throughout this review.