Total internal biliary diversion during liver transplantation for type 1 progressive familial intrahepatic cholestasis: a novel approach
Total internal biliary diversion during liver transplantation for type 1 progressive familial intrahepatic cholestasis: a novel approach
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DOI：
10.1111/petr.12782
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发表时间：
2016-11-01
影响因子：
1.3
通讯作者：
Kasahara, M.
中科院分区：
文献类型：
--
作者：
Mali, V. P.;Fukuda, A.;Kasahara, M.
1型PFIC的LT常并发术后腹泻和移植物脂肪变性复发。一名26个月大的女婴，患有胆汁淤积性黄疸、瘙痒、腹泻和生长迟缓，显示总胆红素9.1 mg/dL、γ-谷氨酰转肽酶64 IU/L和TBA 295.8 μ mol/L。基因分析证实了ATP 8B 1缺陷。对杂合子父亲进行LT（2、3段移植）。在移植肝管和中横结肠之间通过35 cm空肠导管进行胆管分流。粪便立即变黑。第138天的随访显示黄疸和瘙痒消退，软便至硬便（每日6-8次）。放射性同位素肝胆管造影（第26、68和139天）证实胆汁通畅引流至结肠（t（1/2）分别为34、27和19分钟）。第62天的造影剂餐随访证实不存在任何结肠-空肠-肝反流。第140天时，通过胆道支架进行的造影剂随访显示空-结肠吻合通畅，传输满意。移植物活检在LT、138天和9个月的随访中显示了相当程度的大泡性脂肪变性（
LT for PFIC type 1 is often complicated by postoperative diarrhea and recurrent graft steatosis. A 26-month-old female child with cholestatic jaundice, pruritus, diarrhea, and growth retardation revealed total bilirubin 9.1 mg/dL, gamma-glutamyl transpeptidase 64 IU/L, and TBA 295.8 mu mol/L. Genetic analysis confirmed ATP8B1 defects. A LT (segment 2, 3 graft) from the heterozygous father was performed. Biliary diversion was performed by a 35-cm jejunum conduit between the graft hepatic duct and the mid-transverse colon. Stools became pigmented immediately. Follow-up at 138 days revealed resolution of jaundice and pruritus and soft-to-hard stools (6-8 daily). Radioisotope hepato-biliary scintigraphy (days 26, 68, and 139) confirmed unobstructed bile drainage into the colon (t(1/2) 34, 27, and 19 minutes, respectively). Contrast meal follow-through at day 62 confirmed the absence of any colo-jejuno-hepatic reflux. At 140 days, contrast follow-through via the biliary stent revealed patent jejuno-colonic anastomosis and satisfactory transit. Graft biopsy at LT, 138 days, and 9 months follow-up revealed comparable grades of macrovesicular steatosis (