title: Cytomegalovirus-associated biliary atresia: An aetiological and prognostic subgroup.
abstract: OBJECTIVE
  Perinatal cytomegalovirus (CMV) infection is a possible cause or trigger of biliary atresia though clinical evidence is scant. We hypothesised that CMV IgM+ve biliary atresia is a separate clinical entity compared to CMV IgM-ve biliary atresia.
  
  
  METHODS
  Prospective single-centre study. 210 infants with histologically confirmed biliary atresia were treated in our institution (Jan. 2004 to Dec. 2011); of these 20 (9.5%) were CMV IgM+ve at presentation. We compared these with 111 infants who were CMV IgM-ve (controls) for clinical features, biochemistry at presentation and outcome following Kasai portoenterostomy (KPE). A blinded comparison of age-matched liver histology was also performed. Data are quoted as median (interquartile range). A P value ≤ 0.05 was regarded as significant.
  
  
  RESULTS
  Infants with CMV IgM+ve biliary atresia were older at Kasai portoenterostomy (or laparotomy) [70 (60-80) days vs. 56 (44-75)days; P = 0.003] and were more jaundiced [175 (147-224) vs. 140 (121-181) μmol/L; P = 0.002+ with higher AST*287 (157-403) vs. 180 (133-254) IU/L; P = 0.005] and aspartate aminotransferase-to-platelet ratio index [1.1 (0.79-3.0) vs. 0.63 (0.43-0.95)] levels. Liver histology: CMV IgM+ve biliary atresia was characterised by a greater degree of inflammation (P < 0.0001) and fibrosis (P = 0.02), whereas CMV IgM-ve isolated biliary atresia had a higher degree of lobular cholestasis (P = 0.001). This effect was independent of the effects of age at KPE.
  
  
  RESULTS
  CMV IgM+ve biliary atresia had a poorer outcome with a reduced clearance of jaundice (15% vs. 52.2%; P = 0.002), native liver survival (P < 0.0001) and increased mortality (P = 0.002).
  
  
  CONCLUSIONS
  CMV IgM+ve biliary atresia is a distinct clinical and pathological entity with a diminished response to Kasai portoenterostomy.
authors.affiliation: Department of Paediatric Surgery, King's College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, UK.
authors.forename: Augusto
authors.initials: A
authors.lastname: Zani
authors.affiliation: Institute of Liver Studies, King's College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, UK.
authors.forename: Alberto
authors.initials: A
authors.lastname: Quaglia
authors.affiliation: Paediatric Liver Service, King's College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, UK.
authors.forename: Nedim
authors.initials: N
authors.lastname: Hadzić
authors.affiliation: South London Specialist Virology Centre, King's College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, UK.
authors.forename: Mark
authors.initials: M
authors.lastname: Zuckerman
authors.affiliation: Department of Paediatric Surgery, King's College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, UK. Electronic address: Markdav2@ntlworld.com.
authors.forename: Mark
authors.initials: M
authors.lastname: Davenport
mesh_terms: D000367:Age Factors; D001219:Aspartate Aminotransferases / Q000097:blood; D001656:Biliary Atresia / Q000473:pathology / Q000601:surgery / Q000821:virology*; D002779:Cholestasis / Q000209:etiology; D003587:Cytomegalovirus / Q000276:immunology; D003586:Cytomegalovirus Infections / Q000150:complications*; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis; D007223:Infant; D008099:Liver / Q000201:enzymology / Q000473:pathology / Q000601:surgery; D008297:Male; D010976:Platelet Count; D011171:Portoenterostomy, Hepatic; D011379:Prognosis; D011446:Prospective Studies
chemical_list: D007075:Immunoglobulin M; D001219:Aspartate Aminotransferases
keywords: Biliary atresia; Cytomegalovirus (CMV); Kasai portoenterostomy; Neonatal cholestatic jaundice