Takimoto Aitaro, Wolfe Nicholas, Jiahui Liu, Kato Daiki, Yasui Akihiro, Uchida Hiroo, Asai Akihiro
Department of Gastroenterology, Hepatology & Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Department of Pediatric Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan.
J Pediatr Gastroenterol Nutr. 2024 Oct;79(4):818-825. doi: 10.1002/jpn3.12349. Epub 2024 Aug 11.
In patients with biliary atresia (BA), severe portal hypertension (HTN) develops even with successful bile flow restoration, suggesting an intrinsic factor driving portal HTN independent from bile obstruction. We hypothesize that patients with BA have abnormal portal vein (PV) development, leading to PV hypoplasia.
In this observational cohort study, we enrolled patients who were referred to a tertiary center from 2017 to 2021 to rule out BA. Newborns who underwent computed tomography angiogram as a clinical routine before intraoperative cholangiogram, and laparoscopic Kasai hepatoportoenterostomy. The diameter of the PV and hepatic artery (HA) were compared to the degree of liver fibrosis in the wedge biopsies. The jaundice clearance, native liver survival, and clinical portal hypertensive events, including ascites development and intestinal bleeding, were assessed.
47 newborns with cholestasis were included in the cohort; 35 were diagnosed with BA. The patients with BA had a smaller median PV diameter (4.3 vs. 5.1 mm; p < 0.001) and larger median HA diameter (1.4 vs. 1.2 mm; p < 0.05) compared to the patients with other forms of cholestasis. The median PV and HA diameter did not correlate with the degree of liver fibrosis. Among 35 patients with BA, 29 patients (82.9%) achieved jaundice clearance, and 23 patients (65.7%) were alive with their native liver at two years of age. Seven patients (20%) developed intestinal bleeding, and seven patients (20%) developed ascites, with one overlapping patient.
PV hypoplasia is present in patients with BA independent of liver fibrosis at the time of diagnosis.
在胆道闭锁（BA）患者中，即使胆汁流动恢复成功，仍会出现严重的门静脉高压（HTN），这表明存在一种独立于胆汁梗阻驱动门静脉高压的内在因素。我们假设BA患者存在门静脉（PV）发育异常，导致PV发育不全。
在这项观察性队列研究中，我们纳入了2017年至2021年转诊至三级中心以排除BA的患者。在术中胆管造影和腹腔镜Kasai肝门肠吻合术前，作为临床常规进行计算机断层血管造影的新生儿。将PV和肝动脉（HA）的直径与楔形活检中的肝纤维化程度进行比较。评估黄疸清除率、自体肝生存率以及临床门静脉高压事件，包括腹水形成和肠道出血。
队列中纳入了47例胆汁淤积的新生儿；35例被诊断为BA。与其他形式胆汁淤积的患者相比，BA患者的PV中位直径较小（4.3对5.1mm；p<0.001），HA中位直径较大（1.4对1.2mm；p<0.05）。PV和HA的中位直径与肝纤维化程度无关。在35例BA患者中，29例（82.9%）实现了黄疸清除，23例（65.7%）在两岁时自体肝存活。7例（20%）发生肠道出血，7例（20%）发生腹水，有1例重叠患者。
BA患者在诊断时存在PV发育不全，与肝纤维化无关。
