PDF(Pubmed)
Abstract：
UNASSIGNED: Severe pruritus caused by progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (AGS) is refractory to medical treatment. Surgical interruption of the enterohepatic circulation is considered the mainstay of alleviating distressing symptoms and delaying cirrhosis.
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.
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.
摘要：
■由进行性家族性肝内胆汁淤积症（PFIC）和Alagille综合征（AGS）引起的严重瘙痒难以治疗。肝肠循环的手术中断被认为是减轻痛苦症状和延迟肝硬化的主要手段。
■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。
■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。
