The Perinatal Infection of Cytomegalovirus Is an Important Etiology for Biliary Atresia in China

Xu, Yi; Yu, Jian; Zhang, Ruizhong; Yin, Yingxian; Ye, Jiawei; Tan, Limei; Xia, Huimin

doi:10.1177/0009922811406264

Cited by 72 publications

(59 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…One study of disease aetiology in 85 Chinese patients identified cytomegalovirus DNA and/or protein in the livers of 51 infants, 34 and a second study of a smaller cohort showed a low percentage of patients with normalization of bilirubin levels after hepatoportoenterostomy. 35 Poor bile drainage and highest risk of death were also linked to the presence of cytomegalovirus IgM antibodies in a cohort of infants at King's College Hospital, London, UK, who had a 10% survival with the native liver at 2 years of age.…”

Section: Precise Phenotyping Of Diseasementioning

confidence: 99%

Pathogenesis of biliary atresia: defining biology to understand clinical phenotypes

Asai

Miethke

Bezerra

2015

Nat Rev Gastroenterol Hepatol

205

203

View full text Add to dashboard Cite

Biliary atresia is a severe cholangiopathy of early infancy that destroys extrahepatic bile ducts and disrupts bile flow. With a poorly defined disease pathogenesis, treatment consists of the surgical removal of duct remnants followed by hepatoportoenterostomy. Although this approach can improve the short-term outcome, the liver disease progresses to end-stage cirrhosis in most children. Further improvement in outcome will require a greater understanding of the mechanisms of biliary injury and fibrosis. Here, we review progress in the field, which has been fuelled by collaborative studies in larger patient cohorts and the development of cell culture and animal model systems to directly test hypotheses. Advances include the identification of phenotypic subgroups and stages of disease based on clinical, pathological and molecular features. Stronger evidence exists for viruses, toxins and gene sequence variations in the aetiology of biliary atresia, triggering a proinflammatory response that injures the duct epithelium and produces a rapidly progressive cholangiopathy. The immune response also activates the expression of type 2 cytokines that promote epithelial cell proliferation and extracellular matrix production by nonparenchymal cells. These advances provide insight into phenotype variability and might be relevant to the design of personalized trials to block progression of liver disease.

show abstract

Section: Precise Phenotyping Of Diseasementioning

confidence: 99%

Pathogenesis of biliary atresia: defining biology to understand clinical phenotypes

Asai

Miethke

Bezerra

2015

Nat Rev Gastroenterol Hepatol

205

203

View full text Add to dashboard Cite

show abstract

“…CMV has been implicated in neonatal hepatitis [47], ischemic vasculopathy [48], and intrahepatic bile duct paucity [49]. In a recent study in China, CMV DNA was identified in 60% BA patients at the time of diagnosis [50]. A higher rate of jaundice, cholangitis, and degree of liver fibrosis after HPE in BA infants with CMV infection suggests that CMV infection may correlate with a worse prognosis [51].…”

Section: Theories Of Pathogenesismentioning

confidence: 99%

Update on investigations pertaining to the pathogenesis of biliary atresia

Kilgore

Mack

2017

Pediatr Surg Int

View full text Add to dashboard Cite

Biliary atresia is a devastating biliary disease of neonates that results in liver transplantation for the vast majority. The etiology of biliary atresia is unknown and is likely multifactorial, with components of genetic predisposition, environmental trigger and autoimmunity contributing to disease pathogenesis. This review highlights recent work related to investigations of disease pathogenesis in biliary atresia.

show abstract

“…[25] In contrast, a Chinese study demonstrated a lower rate of clearance of jaundice, a higher incidence of cholangitis, and a more severe degree of liver fibrosis and inflammation after Kasai in BA infants with a history of perinatal CMV infection, suggesting that CMV infection may correlate with a worse prognosis. [26] Strong evidence for a perinatal CMV infection associated with BA was recently described by Xu et al[27] Liver tissue obtained at the time of portoenterostomy on 85 infants with BA was analyzed by real-time PCR for the presence of the following viruses: HSV, EBV, VZV, CMV and adenovirus. The majority of the specimens were positive for CMV (51 of 85; 60%) and a few were positive for adenovirus (5/85) and EBV (3/85).…”

Section: Perinatal Virus Infection and Biliary Atresiamentioning

confidence: 99%

“…Studies confirming the presence of CMV in PCR positive liver samples were performed with immunocytochemical detection of CMV-pp65 antigens within liver bile duct epithelia and hepatocytes. [27]…”

Section: Perinatal Virus Infection and Biliary Atresiamentioning

confidence: 99%

Biliary atresia: cellular dynamics and immune dysregulation

Feldman

Mack

2012

Seminars in Pediatric Surgery

View full text Add to dashboard Cite

The cause of biliary atresia (BA) is unknown and in the past few decades the majority of investigations related to pathogenesis have centered on virus infections and immunity. The acquired or perinatal form of BA entails a progressive, inflammatory injury of bile ducts, leading to fibrosis and obliteration of both the extrahepatic and intrahepatic bile ducts. Theories of pathogenesis include viral infection, chronic inflammatory or autoimmune-mediated bile duct injury and abnormalities in bile duct development. This review will focus solely on human studies pertaining to a potential viral trigger of bile duct injury at diagnosis and provide insight into the interplay of the innate and adaptive immune responses in the pathogenesis of disease.

show abstract

The Perinatal Infection of Cytomegalovirus Is an Important Etiology for Biliary Atresia in China

Abstract: Human intrahepatic biliary epithelial cell is the target cell of HCMV. The etiology of biliary atresia is probably multifactorial. The perinatal infection of HCMV is one of the important etiologies for biliary atresia in China.

Cited by 72 publications

References 20 publications

Pathogenesis of biliary atresia: defining biology to understand clinical phenotypes

Pathogenesis of biliary atresia: defining biology to understand clinical phenotypes

Update on investigations pertaining to the pathogenesis of biliary atresia

Biliary atresia: cellular dynamics and immune dysregulation

Contact Info

Product

Resources

About