Abstract
BackgroundBiliary atresia (BA) is a neonatal cholestatic disease of unknown etiology. It is the leading cause of liver transplantation in children. Many similarities exist between BA and graft versus host disease suggesting engraftment of maternal cells during gestation could result in immune responses that lead to BA. The aim of this study was to determine the presence and extent of maternal microchimerism (MM) in the livers of infants with BA.MethodsUsing fluorescent in situ hybridization (FISH), 11 male BA & 4 male neonatal hepatitis (NH) livers, which served as controls, were analyzed for X and Y-chromosomes. To further investigate MM in BA, 3 patients with BA, and their mothers, were HLA typed. Using immunohistochemical stains, the BA livers were examined for MM. Four additional BA livers underwent analysis by polymerase chain reaction (PCR) for evidence of MM.ResultsBy FISH, 8 BA and 2 NH livers were interpretable. Seven of eight BA specimens showed evidence of MM. The number of maternal cells ranged from 2–4 maternal cells per biopsy slide. Neither NH specimen showed evidence of MM. In addition, immunohistochemical stains confirmed evidence of MM. Using PCR, a range of 1–142 copies of maternal DNA per 25,000 copies of patients DNA was found.ConclusionsMaternal microchimerism is present in the livers of patients with BA and may contribute to the pathogenesis of BA.
Highlights
Biliary atresia (BA) is a neonatal cholestatic disease of unknown etiology
Detection of female cells in male BA liver biopsies Using fluorescent in situ hybridization (FISH), we examined 11 male BA liver biopsies and 4 male neonatal hepatitis (NH) liver biopsies
That maternal microchimerism may play a role in the etio-pathogenesis of BA by causing an alloimmune reaction given the similarities between BA and Graft versus host disease (GVHD)
Summary
Biliary atresia (BA) is a neonatal cholestatic disease of unknown etiology. It is the leading cause of liver transplantation in children. [1,2,3] Both the intra-and extra-hepatic biliary ducts demonstrate evidence of a progressive destruction. This results in cholestasis, hepatic fibrosis and eventually cirrhosis. BA is associated with significant morbidity and mortality. The incidence of BA is 1 in 10,000 to 14,000 live births, [4,5] it accounts for over 40% of the neonatal cholestatic liver disease in Europe and the United States. Two leading hypothesis are that BA occurs as a result of ductal plate malformations occurring during development of the liver, or as a result of an immune mediated process triggered by yet to be determined stimulus. [9] As more knowledge accrues about the histology and immunologic characteristics of BA, the more it appears to be a progressive immune mediated process
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