Foroutan Hamid Reza, Bahador Ali, Ghanim Sultan Mohsin, Dehghani Seyed Mohsen, Anbardar Mohammad Hossein, Fattahi Mohammad Reza, Forooghi Mehdi, Azh Omidreza, Tadayon Ali, Sherafat Alireza, Yaghoobi Amir Arsalan, Ashraf Mohammad Ali
Laparoscopy Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Department of Pediatric Surgery, Nemazee Hospital, Shiraz University of Medical Sciences, Zand St., 71 32326645, Shiraz, Iran.
Pediatr Surg Int. 2020 May;36(5):603-610. doi: 10.1007/s00383-020-04641-z. Epub 2020 Mar 23.
Progressive familial intrahepatic cholestasis (PFIC) is a hereditary disease characterized by cholestasis, which may cause jaundice, severe pruritus, and cirrhosis in the later stages. By the invention of biliary diversion methods, these patients were prevented from undergoing liver transplant. Using biliary diversion techniques, the entero-hepatic cycle was interrupted. This lowers the bile acid pool and resolves the pruritus. Herein, we report 44 cases of PFIC who underwent partial internal biliary diversion (PIBD) and long-term follow-up of these children. This comprises the largest case series of PIBD.
All patients were diagnosed by liver biopsy as PFIC before the operation. All underwent cholecysto colic bypass by jejunal interposition due to severe pruritus unresponsive to medication. Laboratory blood tests, sonography, and physical exam were done before and after the operation once every 3 months. Besides, a questionnaire was designed to ask the patients about the symptoms after the operation, and a pruritus score was measured using the 5D-itch scale.
44 children (25 boys, 19 girls), between 1.75 and 27.5 years (at the time of this study) were followed for a median period of 54 months. Age at operation ranged from 2 months to 18 years, with a median of 29 months. Of these children, 14 were lost to follow up. Results showed a significant decrease in pruritus and sleep disturbance after the surgery (p < 0.001). Also, jaundice decreased from 82.1 before to 7.1% following the surgery. 50% of the patients became medication-free at follow-up.
PIBD is a safe procedure which helps non-cirrhotic children preserve their liver function. Therefore, PIBD prevents them from undergoing liver transplant. Effective results were achieved in terms of severe pruritus and jaundice, and children were able to regain their sleep patterns. It also avoided external stoma, which is more convenient from the patient's point of view.
进行性家族性肝内胆汁淤积症（PFIC）是一种以胆汁淤积为特征的遗传性疾病，后期可能导致黄疸、严重瘙痒和肝硬化。通过发明胆汁转流方法，这些患者得以避免进行肝移植。使用胆汁转流技术，肠肝循环被中断。这降低了胆汁酸池并缓解了瘙痒。在此，我们报告44例接受部分肝内胆汁转流术（PIBD）的PFIC患儿及其长期随访情况。这是最大的PIBD病例系列。
所有患者术前均经肝活检确诊为PFIC。由于药物治疗无效的严重瘙痒，所有患者均通过空肠间置进行胆囊结肠旁路术。术前和术后每3个月进行一次实验室血液检查、超声检查和体格检查。此外，设计了一份问卷询问患者术后症状，并使用5D瘙痒量表测量瘙痒评分。
44名儿童（25名男孩，19名女孩），年龄在1.75至27.5岁之间（本研究时），中位随访期为54个月。手术年龄从2个月至18岁不等，中位年龄为29个月。这些儿童中，14名失访。结果显示术后瘙痒和睡眠障碍显著减轻（p<0.001）。黄疸也从术前的82.1%降至术后的7.1%。50%的患者在随访时无需用药。
PIBD是一种安全的手术，有助于非肝硬化儿童保留肝功能。因此，PIBD可防止他们进行肝移植。在严重瘙痒和黄疸方面取得了有效结果，儿童能够恢复睡眠模式。它还避免了外置造口，从患者角度来看更方便。
