Incidence, Impact and Treatment of Ongoing CMV Infection in Patients with Biliary Atresia in Four European Centres.

Björn Fischler,Piotr Socha,Ulrika Liliemark,Ronald J Sokol,Antal Dezsofi,Mark Davenport,Piotr Czubkowski,Jan F Svensson

doi:10.3390/jcm11040945

Abstract

Cytomegalovirus (CMV) infection has been suggested to be of importance for the development and outcome of biliary atresia (BA). However, most data are only available from single centre studies. We retrospectively collected data on rates, outcomes, and treatments for ongoing CMV infection at the time of Kasai portoenterostomy (KPE) from four different tertiary centres in Europe. The rate of ongoing CMV infection varied between 10–32% in the four centres. CMV positive patients were significantly older and had higher levels of several liver biochemistries at the time of KPE (p < 0.05 for all comparisons). In the largest centre, CMV infection was more common in non-Caucasians, and CMV infected patients had poorer long-term survival with native liver than CMV negative patients (p = 0.0001). In contrast, survival with native liver in the subgroup of CMV infected patients who had received antiviral treatment was similar to the CMV negative group. We conclude that ongoing CMV infection at the time of KPE occurs in a significant proportion of BA patients and that these patients seem to differ from CMV negative patients regarding age and biochemistry at the time of KPE as well as long-term survival with native liver. The latter difference may be reduced by antiviral treatment, but randomized, controlled trials are needed before such treatment can be recommended routinely.

Highlights

Cytomegalovirus (CMV) is a double-stranded DNA virus of the family herpesviridae.Though infection is normally mild in children and adults it can be serious in the immunocompromised host
In an early Swedish study from 1998, CMVIgM was detected in serum from 38% of biliary atresia (BA) patients at the time of Kasai portoenterostomy (KPE), which was significantly higher than the 6% found in age-matched controls without any liver disease [1]
By collecting and comparing multicentre data from four tertiary centres we showed that the rate of CMV positivity in BA patients varies between centres, but that it is significantly higher than expected for age [1]

Summary

Introduction

Cytomegalovirus (CMV) is a double-stranded DNA virus of the family herpesviridae.Though infection is normally mild in children and adults it can be serious in the immunocompromised host. In an early Swedish study from 1998, CMVIgM was detected in serum from 38% of BA patients at the time of Kasai portoenterostomy (KPE), which was significantly higher than the 6% found in age-matched controls without any liver disease [1]. Other studies have since suggested the rate of BA patients with ongoing CMV infection to be anywhere between 10 and 74% with Asian centres tending to have a higher prevalence [2,3,4,5,6]. In the largest European study to date, Zani et al showed that infants with “CMV-IgM +ve BA” were older at the time of KPE and had distinctively different histopathological features in the liver, including more pronounced inflammation than BA patients without CMV infection [3]. It was reported that CMV IgM +ve patients had a worse prognosis that was improved following anti-viral treatment (AVT) [7]

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