Department of Pediatrics, University of Zagreb Medical School, University Hospital Centre Zagreb, Zagreb, Croatia.
Eur J Pediatr. 2012 Dec;171(12):1799-804. doi: 10.1007/s00431-012-1820-7. Epub 2012 Sep 15.
The presence of ductal plate malformation (DPM+) on liver histology in children with biliary atresia (BA) is a marker of early intrauterine disease onset and an indication of an unfavorable prognosis. We studied the prognostic value of DPM in infants with BA after hepatoportoenterostomy (HPE). We reviewed 28 BA patients who underwent HPE in a single medical center. We examined the time of jaundice onset after delivery (conjugated hyperbilirubinemia): early onset (fetal phenotype with no jaundice-free interval) vs. late onset (perinatal phenotype with jaundice-free interval) and the presence or absence of DPM (DPM+ or DPM-) histopathology. Primary outcome was jaundice clearance at 3 months after HPE and survival with native liver (SNL). Eight children had fetal and 20 had perinatal BA (8 DPM+, 12 DPM-). At 3 months after HPE, no patients with fetal BA had achieved jaundice clearance, while jaundice clearance was achieved in five patients with DPM+ perinatal disease and four patients with DPM- perinatal BA (P = 0.03, comparing all three groups; P = 0.36, comparing DPM+ vs. DPM- perinatal patients). Median SNL was 8.6 months for fetal BA patients, 148.2 months for DPM+ perinatal BA patients, and 93.2 months for DPM- perinatal BA patients (log-rank test, P < 0.001, comparing all three groups; P = 0.59, comparing DPM+ vs. DPM- perinatal patients). After adjusting for BA type, age older than 2 months at HPE was associated with worse SNL [P = 0.03; hazard ratio = 4.0 (95 % CI, 1.1-14.2)].
Early onset of jaundice, regardless of DPM histology, was the most ominous sign of poor outcome in infants with BA after HPE.
在胆道闭锁（BA）患儿的肝脏组织学中存在胆管板畸形（DPM+）是宫内疾病起始较早的标志，也是预后不良的指征。我们研究了 DPM 在接受肝门空肠吻合术（HPE）后的 BA 婴儿中的预后价值。我们回顾了在单一医疗中心接受 HPE 的 28 名 BA 患者。我们检查了分娩后黄疸出现的时间（结合胆红素升高）：早发（无黄疸间歇的胎儿表型）与晚发（有黄疸间歇的围生期表型），以及 DPM 的存在与否（DPM+或 DPM-）组织病理学。主要结局是 HPE 后 3 个月时黄疸消退和具有原生肝的存活率（SNL）。8 名患儿为胎儿型，20 名患儿为围生期型（8 名 DPM+，12 名 DPM-）。在 HPE 后 3 个月时，没有胎儿 BA 患儿的黄疸消退，而 DPM+围生期疾病的 5 名患儿和 DPM-围生期 BA 的 4 名患儿黄疸消退（所有三组之间比较，P=0.03；DPM+与 DPM-围生期患者比较，P=0.36）。胎儿 BA 患儿的中位 SNL 为 8.6 个月，DPM+围生期 BA 患儿为 148.2 个月，DPM-围生期 BA 患儿为 93.2 个月（对数秩检验，所有三组之间比较，P<0.001；DPM+与 DPM-围生期患者比较，P=0.59）。调整 BA 类型后，HPE 时年龄大于 2 个月与 SNL 较差相关[P=0.03；风险比=4.0（95%CI，1.1-14.2）]。
HPE 后 BA 婴儿中，无论 DPM 组织学如何，黄疸早发是预后不良的最严重征象。
