UNASSIGNED: This study aims to evaluate the short-term effect of partial external biliary diversion (PEBD) on pruritus, liver disease progression, patient\'s growth, and quality of life.
UNASSIGNED: This prospective cohort study enrolled children with PFIC and AGS from July 2019 to July 2021, whose guardians consented to the PEBD procedure. A standard surgical approach was performed by a single surgeon. Outcomes were measured subjectively and objectively pre- and post-procedure using the pruritus 5-D itching score, Paediatric Quality of Life Inventory scale (PedsQL), growth parameters, bile acids level, and liver function tests. Patients\' follow-up period ranged from 6 to 12 months.
UNASSIGNED: Seven patients had PEBD procedure; five with PFIC and two with AGS. A significant improvement was detected in the 5-D itching score (p-value < 0.001), PedsQL (p-value < 0.001), and bile acids level (p-value 0.013). The preexisting growth failure was ameliorated. The downward trend in the bilirubin level was not significant. No influential difference in the other liver function tests occurred. No intra-operative complications encountered. Only one case had a post-operative stoma prolapse which was managed surgically.
UNASSIGNED: PEBD procedure could be considered as an effective and safe treatment options for intractable pruritus in patients with PFIC or AGS, providing preserved synthetic liver functions.
■本研究旨在评估部分外部胆道改道(PEBD)对瘙痒的短期影响,肝脏疾病进展,病人的成长,和生活质量。
这项前瞻性队列研究纳入了2019年7月至2021年7月的PFIC和AGS儿童,其监护人同意PEBD程序。由一名外科医生进行标准手术方法。使用瘙痒5-D瘙痒评分在手术前后主观和客观地测量结果,儿科生活质量量表(PedsQL),生长参数,胆汁酸水平,和肝功能测试.患者的随访期为6至12个月。
■7名患者接受了PEBD手术;5名患者接受了PFIC,2名患者接受了AGS。在5-D瘙痒评分(p值<0.001)中检测到显着改善,PedsQL(p值<0.001),和胆汁酸水平(p值0.013)。先前存在的生长失败得到了改善。胆红素水平下降趋势不显著。其他肝功能检查无影响差异。术中无并发症。只有一例手术后的造口脱垂通过手术治疗。
■PEBD手术可被认为是PFIC或AGS患者顽固性瘙痒的有效和安全的治疗选择,提供保留的合成肝功能。