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自身免疫性肝炎的肝移植:单中心经验

Liver transplantation for autoimmune hepatitis: a single-center experience.

作者信息

Khalaf H, Mourad W, El-Sheikh Y, Abdo A, Helmy A, Medhat Y, Al-Sofayan M, Al-Sagheir M, Al-Sebayel M

机构信息

Department of Liver Transplantation and Hepatobiliary-Pancreatic Surgery, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.

出版信息

Transplant Proc. 2007 May;39(4):1166-70. doi: 10.1016/j.transproceed.2007.02.030.

Abstract

OBJECTIVE

To present our experience with deceased donor liver transplantation (DDLT) and living-donor liver transplantation (LDLT) for autoimmune hepatitis (AIH).

PATIENTS AND METHOD

Between April 2001 and November 2006, a total of 116 LT procedures were performed (73 DDLTs and 43 LDLTs) in 112 patients (4 retransplants). Of the 112 recipients, 16 patients (14.3%) were transplanted for AIH (15 DDLTs and 1 LDLT). All recipients received FK506- and steroid-based immunosuppressive regimens.

RESULTS

The male/female ratio was 3/13, median age was 22 years (range, 15 to 35), and the median MELD score was 25 (range, 11 to 40). Arterial reconstruction was needed in four DDLTs due to severe steroid-induced angiopathy. After a median follow-up period of 530 days (range, 11 to 2016), the overall patient and graft survival rates were 93.8%. Only one patient died following LDLT due to primary graft nonfunction. Histopathologic recurrence was seen in three patients (18.7%) and was successfully treated by optimizing immunosuppression. Markedly elevated serum CA19-9 levels (median, 1069; range, 217 to 2855) was seen in four patients (28%), malignancy was ruled out and all patients normalized serum CA19-9 levels within the first 3 months posttransplant. Steroids withdrawal failed in all recipients and was always accompanied with almost immediate elevation of liver enzymes.

CONCLUSIONS

In our experience, LT for AIH shows excellent long-term outcomes, patients are usually young women who present with acute deterioration and high MELD scores, and usually require long-term steroids to prevent rejection and disease recurrence. Some patients have markedly high CA19-9 in absence of malignancy. Some patients also have severe steroid-induced hepatic artery angiopathy necessitating arterial reconstruction during the transplant surgery.

摘要

目的

介绍我们在自身免疫性肝炎(AIH)患者中进行尸体供肝肝移植(DDLT)和活体供肝肝移植(LDLT)的经验。

患者与方法

2001年4月至2006年11月期间,共对112例患者(4例再次移植)实施了116例肝移植手术(73例DDLT和43例LDLT)。在这112例受者中,16例患者(14.3%)因AIH接受移植(15例DDLT和1例LDLT)。所有受者均接受基于FK506和类固醇的免疫抑制方案。

结果

男女比例为3/13,中位年龄为22岁(范围15至35岁),中位终末期肝病模型(MELD)评分25分(范围11至40分)。4例DDLT因严重的类固醇诱导血管病变需要进行动脉重建。中位随访期530天(范围11至2016天)后,总体患者和移植物生存率为93.8%。仅1例LDLT受者因原发性移植物无功能死亡。3例患者(18.7%)出现组织病理学复发,通过优化免疫抑制成功治疗。4例患者(28%)血清CA19-9水平显著升高(中位值1069;范围217至2855),排除恶性肿瘤,所有患者在移植后前3个月内血清CA19-9水平恢复正常。所有受者停用类固醇均失败,且总是几乎立即伴有肝酶升高。

结论

根据我们的经验,AIH患者的肝移植显示出良好的长期疗效,患者通常为年轻女性,表现为急性恶化且MELD评分高,通常需要长期使用类固醇以预防排斥反应和疾病复发。一些患者在无恶性肿瘤的情况下CA19-9显著升高。一些患者还患有严重的类固醇诱导肝动脉血管病变,需要在移植手术期间进行动脉重建。

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