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Cytomegalovirus-associated biliary atresia: An aetiological and prognostic subgroup
Augusto Zani1, Alberto Quaglia2, Nedim Hadzić3
1Department of Paediatric Surgery, King's College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, UK.
Background And Aims:
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.
Outcome:
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.
Insights
Cytomegalovirus (CMV) IgM-positive biliary atresia is a distinct condition from CMV IgM-negative biliary atresia. Infants with CMV IgM-positive biliary atresia show poorer outcomes and a reduced response to Kasai portoenterostomy.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Infectious Diseases
Background:
- Perinatal cytomegalovirus (CMV) infection is a suspected, yet poorly evidenced, cause of biliary atresia.
- This study investigates whether CMV IgM-positive biliary atresia represents a distinct clinical entity compared to CMV IgM-negative biliary atresia.
Purpose of the Study:
- To determine if cytomegalovirus (CMV) IgM-positive biliary atresia is a separate clinical and pathological entity.
- To compare the clinical features, biochemical markers, liver histology, and treatment outcomes of CMV IgM-positive versus CMV IgM-negative biliary atresia.
Main Methods:
- A prospective, single-center study included 210 infants with confirmed biliary atresia.
- 20 infants were CMV IgM-positive and compared with 111 CMV IgM-negative infants (controls).
- Evaluated clinical presentation, biochemistry, outcomes post-Kasai portoenterostomy (KPE), and performed blinded histological analysis.
Main Results:
- CMV IgM-positive biliary atresia cases presented at an older age, with higher jaundice levels and AST.
- Histological analysis revealed greater inflammation and fibrosis in CMV IgM-positive cases, contrasting with increased lobular cholestasis in CMV IgM-negative cases.
- Outcomes were poorer for CMV IgM-positive biliary atresia, including reduced jaundice clearance, native liver survival, and increased mortality.
Conclusions:
- CMV IgM-positive biliary atresia is a distinct clinical and pathological entity.
- This condition is associated with a diminished response to Kasai portoenterostomy.
- Findings suggest CMV plays a significant role in the pathogenesis of a subset of biliary atresia cases.
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