Department of Gastroenterology, Hepatology and Clinical Nutrition, The Royal Children's Hospital Melbourne, Melbourne, Victoria, Australia.
Department of Pediatrics, All India Institute of Medical Sciences, Rishikesh, India.
J Paediatr Child Health. 2022 Jul;58(7):1221-1227. doi: 10.1111/jpc.15954. Epub 2022 Apr 4.
Primary sclerosing cholangitis (PSC) is a chronic progressive cholestatic disorder associated with ulcerative colitis (UC). Although the inflammatory bowel disease phenotype has been characterised in patients with PSC, the impact of UC on the course and progression of PSC-UC is less clear. We aimed to evaluate the effects of UC on liver-related outcomes in children with PSC.
Retrospective analysis of children aged ≤18 years diagnosed with PSC with/without UC at a single tertiary paediatric liver unit between January 1998 and May 2016. Patients were followed up until transition to an adult service. Outcomes studied included biliary complications, clinically significant portal hypertension, need for liver transplantation and post-transplantation recurrence.
Fifty-one children (31 female) were diagnosed with PSC (median age - 11.3 years (interquartile range 7)), follow-up median duration 54 months (interquartile range 56). Thirty-seven (73%) patients had concurrent UC, of which 26 had their diagnosis confirmed prior to or within 6 months of PSC diagnosis (early-onset). PSC complications were more common in children with PSC-UC compared with PSC alone (24/37 (65%) vs. 2/14 (14%); P = 0.001). Furthermore, children with endoscopically mild or moderate UC at diagnosis showed a greater propensity for liver-related complications compared with children with severe UC (24/32 vs. 0/5; P = 0.003). Children with late-onset UC had higher rates of clinically significant portal hypertension (5/11 (45%) vs. 3/26 (12%); P = 0.007) and liver transplantation (5/11(45%) vs. 2/26 (8%); P = 0.02). Children with PSC-UC had significantly higher rates of pancolitis, rectal sparing and milder colitis than those with UC alone.
The presence and a later-onset of UC are associated with more significant progression to end-stage liver disease. There is an inverse trend between UC severity and PSC severity in children with concurrent PSC-UC.
原发性硬化性胆管炎（PSC）是一种与溃疡性结肠炎（UC）相关的慢性进行性胆汁淤积性疾病。尽管 PSC 患者的炎症性肠病表型已得到描述，但 UC 对 PSC-UC 病程和进展的影响尚不清楚。我们旨在评估 UC 对儿童 PSC 患者肝脏相关结局的影响。
对 1998 年 1 月至 2016 年 5 月期间在一家三级儿科肝脏专科接受 PSC 合并/不合并 UC 诊断的≤18 岁儿童进行回顾性分析。患者随访至过渡至成人服务。研究的结局包括胆道并发症、临床显著门静脉高压、需要肝移植和移植后复发。
51 名儿童（31 名女性）被诊断为 PSC（中位数年龄为 11.3 岁（四分位距 7）），中位随访时间为 54 个月（四分位距 56）。37 名（73%）患者合并 UC，其中 26 名在 PSC 诊断前或诊断后 6 个月内确诊（早发性）。与单纯 PSC 相比，PSC-UC 患儿更常见 PSC 并发症（24/37（65%）vs. 2/14（14%）；P=0.001）。此外，诊断时内镜下轻度或中度 UC 的患儿发生肝脏相关并发症的倾向性大于重度 UC 患儿（24/32 与 0/5；P=0.003）。晚发性 UC 患儿的临床显著门静脉高压（5/11（45%）vs. 3/26（12%）；P=0.007）和肝移植（5/11（45%）与 2/26（8%）；P=0.02）发生率更高。PSC-UC 患儿的全结肠炎、直肠保留和轻度结肠炎发生率明显高于 UC 单独患儿。
UC 的存在和晚发性与终末期肝病的更显著进展相关。在合并 PSC-UC 的儿童中，UC 严重程度与 PSC 严重程度呈负相关。
