Zani Augusto, Quaglia Alberto, Hadzić Nedim, Zuckerman Mark, Davenport Mark
Department of Paediatric Surgery, King's College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, UK.
Institute of Liver Studies, King's College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, UK.
J Pediatr Surg. 2015 Oct;50(10):1739-45. doi: 10.1016/j.jpedsurg.2015.03.001. Epub 2015 Mar 7.
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.
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.
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.
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).
CMV IgM+ve biliary atresia is a distinct clinical and pathological entity with a diminished response to Kasai portoenterostomy.
尽管临床证据不足，但围产期巨细胞病毒（CMV）感染可能是胆道闭锁的一个病因或触发因素。我们推测，与CMV IgM阴性的胆道闭锁相比，CMV IgM阳性的胆道闭锁是一种独立的临床实体。
前瞻性单中心研究。2004年1月至2011年12月期间，我们机构对210例经组织学确诊的胆道闭锁婴儿进行了治疗；其中20例（9.5%）就诊时CMV IgM呈阳性。我们将这些患儿与111例CMV IgM阴性的婴儿（对照组）在临床特征、就诊时的生化指标以及Kasai肝门空肠吻合术（KPE）后的结局方面进行了比较。还对年龄匹配的肝脏组织学进行了盲法比较。数据以中位数（四分位间距）表示。P值≤0.05被视为具有统计学意义。
CMV IgM阳性的胆道闭锁婴儿在接受Kasai肝门空肠吻合术（或剖腹手术）时年龄更大[70（60 - 80）天 vs. 56（44 - 75）天；P = 0.003]，黄疸更严重[175（147 - 224） vs. 140（121 - 181）μmol/L；P = 0.002]，天冬氨酸转氨酶（AST）水平更高[287（157 - 403） vs. 180（133 - 254）IU/L；P = 0.005]，天冬氨酸转氨酶与血小板比值指数也更高[1.1（0.79 - 3.0） vs. 0.63（0.43 - 0.95）]。肝脏组织学：CMV IgM阳性的胆道闭锁特征为炎症程度更高（P < 0.0001）和纤维化程度更高（P = 0.02），而CMV IgM阴性的孤立性胆道闭锁小叶胆汁淤积程度更高（P = 0.001）。这种影响独立于KPE时的年龄影响。
CMV IgM阳性的胆道闭锁结局较差，黄疸清除率降低（15% vs. 52.2%；P = 0.002），自体肝存活率降低（P < 0.0001），死亡率增加（P = 0.002）。
CMV IgM阳性的胆道闭锁是一种独特的临床和病理实体，对Kasai肝门空肠吻合术的反应较差。
