Toshihiro Muraji published latest article in Pediatric surgery international entitled Unique manifestations of biliary atresia provide new immunological insight into its etiopathogenesis. This article is available in PubMed with an unique identification number PMID: 29022092 and it is published in 2017. The coauthors of this article are Muraji T, Ohtani H, Ieiri S.
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Article Title: Unique manifestations of biliary atresia provide new immunological insight into its etiopathogenesis.
Co-Author(s): Muraji, T; Ohtani, H; Ieiri, S
Affiliation(s): Department of Pediatric Surgery, Kirishima Medical Center, Kirishima, Japan. email@example.com.
PMID 29022092, Year 2017
Abstract: Biliary atresia (BA) is a unique cholestatic disease of newborns with a background of exaggerated immune response in the liver of unknown mechanism. Three hypotheses have been proposed; autoimmune type of cholangiopathy triggered by virus infection, graft-versus-host disease type of immune-mediated disease associated with maternal microchimerism and ductal plate malformation theory. Researchers on virus infection theory have experimentally explored immune process causing cholangiopathy on murine models of this disease, while in maternal microchimerism hypothesis were detected maternal cells in the BA patients liver, of which roles are yet to be determined. Ductal plate malformation theory is an intriguing hypothesis in the sense that it suggests the onset of this disease is in the first trimester. This theory can be secondary to either one of these two immune-related insults. In this review, four unique points are focused; (1) the timing of onset, (2) hepatitis-like pathological picture, (3) heterogenous atrophy of the liver segments when advanced, and (4) swollen lymph nodes at the porta hepatis. These unique clinicopahtological aspects of this disease should be well explained by these hypotheses.
Journal: Pediatric surgery international