Allograft Steatohepatitis in Progressive Familial Intrahepatic Cholestasis Type 1 After Living Donor Liver Transplantation
Allograft Steatohepatitis in Progressive Familial Intrahepatic Cholestasis Type 1 After Living Donor Liver Transplantation
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DOI：
10.1002/lt.21686
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发表时间：
2009-06-01
影响因子：
4.6
通讯作者：
Uemoto, Shinji
中科院分区：
文献类型：
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作者：
Miyagawa-Hayashino, Aya;Egawa, Hiroto;Uemoto, Shinji
我们研究了肝移植 (LT) 后进行性家族性肝内胆汁淤积 1 型 (PFIC1) 患者的组织学特征和长期结局。组织学结果与 11 名 PFIC1 受体的 LT 后病程和治疗相关。接受 LT 的年龄从 1 岁到 18 岁不等（中位数为 4 岁）。 8 名患者在 LT 后平均 60 天（范围为 21-191 天）观察到大泡脂肪变性。 7 名患者的严重脂肪变性在中位 161 天（范围：116-932 天）内进展为脂肪性肝炎。患者的随访时间中位数为 7.3 年（范围为 2.3-16.1 年）。 6 例出现桥接性纤维化，其中 2 例进展为肝硬化。一名肝硬化患者在 LT 后 13.6 年因脾动脉瘤破裂而死亡。所有 8 名脂肪变性患者均出现 LT 后难治性腹泻。三个没有 LT 后腹泻的患者没有表现出同种异体移植脂肪变性。胆汁吸附树脂疗法减少了腹泻和脂肪变性。移植后脂肪变性患者通常有更严重的 ATPase 1 类 8B 成员 1 (ATP8B1) 基因突变，并且更有可能出现胰腺炎等全身并发症。总之，PFIC1 患者存在同种异体移植脂肪变性，并进展为脂肪性肝炎和肝硬化。由于家族性肝内胆汁淤积 1 基因在多个器官中表达，包括小肠、胰腺和肝脏，并且参与肠肝胆汁酸循环，因此 LT 后脂肪变性可能是由于 ATP8B1 产物的故障所致。肝脏移植 15:610-618, 2009。(C) 2009 AASLD。
We studied histological features and long-term outcomes in patients with progressive familial intrahepatic cholestasis type 1 (PFIC1) after liver transplantation (LT). Histological findings were correlated with the post-LT course and treatment in 11 recipients with PFIC1. Ages at LT varied from 1 to 18 years (median, 4 years). Macrovesicular steatosis was observed in 8 patients at a median of 60 days post-LT (range, 21-191 days). Severe steatosis progressed to steatohepatitis in 7 patients at a median of 161 days (range, 116-932 days). The patients were followed up for a median of 7.3 years (range, 2.3-16.1 years). Six showed bridging fibrosis, with 2 progressing to cirrhosis. One patient with cirrhosis died because of the rupture of a splenic artery aneurysm 13.6 years post-LT. Post-LT refractory diarrhea was present in all 8 having steatosis. Three without post-LT diarrhea showed no allograft steatosis. Bile adsorptive resin therapy reduced the diarrhea and steatosis. Patients with posttransplant steatosis typically had more severe mutations of the ATPase class 1 type 8B member 1 (ATP8B1) gene and were more likely to have systemic complications such as pancreatitis. In conclusion, allograft steatosis was present in patients with PFIC1, progressing to steatohepatitis and cirrhosis. Because expression of the familial intrahepatic cholestasis 1 gene occurs in several organs, including the small intestine, pancreas, and liver, and it is involved in enterohepatic bile acid circulation, post-LT steatosis may be due to a malfunction of the ATP8B1 product. Liver Transpl 15:610-618, 2009. (C) 2009 AASLD.