Problems related to CMV infection and biliary atresia

Abstract

Human cytomegalovirus (CMV) infection is related to biliary disease, being cholestatic in its own right. It has also been associated with intrahepatic bile duct destruction and duct paucity, indicating a possible role in extrahepatic biliary atresia pathogenesis and progression. When related to biliary atresia CMV IGM positive patients appear to have more liver damage thus affecting outcome. Methods We carried out a retrospective chart review on 74 patients diagnosed with hepatobiliary disease (2000-2011). included clinical and outcome review as well as evaluation of potential risk factors. Patients were divided into 2 groups those with biliary atresia and those without Biliary atresia (BA). The 2 groups were compared in terms of CMV infection. Of the 74 patients with hepatobiliary disease investigated, 39 (52%) were shown to have Biliary atresia and 35 other cases. 12 of the BA group and 4 of the non-BA were excluded due to lack of data Twenty-seven (69%) of the biliary atresia group had sufficient available data for review. Of these, 21 (78% of the 27) had CMV positivity (IgM/IgG) on testing, with 20 of these being IgM positive versus 8 in the non-biliary atresia group. (p<0.01) Two (7.5%) of 27 BA infants were HIV exposed being born to HIV positive mothers whereas HIV positivity was observed in 7 (35%) of the non-biliary atresia group (p<0.01). Both of these biliary atresia infants were CMV IgM positive. Long- term outcome of the 21 with CMV positivity showed 3 deaths (non-HIV exposed) and a higher rate of severe early liver damage suggesting a poorer outcome in CMV affected patients. This study suggests a correlation between CMV exposure, infection and surgical hepatobiliary disease including biliary atresia affecting outcome.HIV positivity does not preclude Biliary atresia and should be further investigated.