Siddiqui A R, Abbas Z, Luck N H, Hassan S M, Aziz T, Mubarak M, Naqvi S A, Rizvi S A H
Department of Hepatogastroenterology, Sindh Institute of Urology and Transplantation, Karachi, Pakistan.
Transplant Proc. 2012 Apr;44(3):721-4. doi: 10.1016/j.transproceed.2011.12.019.
Fibrosing cholestatic hepatitis C (FCH-C) is a rare entity that occurs among immune-compromised patients resulting from the direct hepatotoxicity of a high intracellular viral load along with an ineffective immune system ultimately leading to a fatal outcome. We have describes herein 4 renal transplant recipients who were diagnosed with FCH-C at our institution in the last 8 months.
Four renal transplant recipients presented with jaundice and deteriorating liver function tests. They were diagnosed to display FCH-C based on the presence of hepatitis C virus (HCV) RNA and characteristic liver biopsy findings; there was no evidence of any other cause of cholestasis or biliary obstruction.
The patients were men of ages 40, 25, 20, and 27 years. The durations after transplantation were 1.5, 10, 1.5 and 2.0 years, respectively. In all cases pretransplantation screening was negative for HCV antibody, HCV RNA, and hepatitis B surface antigen (HBsAg). All 4 patients were infected with genotype 1, whereas case 2 had coinfection with type 3. Cases 1 and 2 who were treated with interferon and ribavirin, showed improvement in cholestasis but did not achieve a rapid virological response. Case 1 developed graft dysfunction secondary to acute cellular rejection at 4 months after initiation of interferon treatment, which was treated with pulse steroids. Interferon-based therapy was stopped prematurely in both cases due to pancytopenia. Case 3 developed florid pyelonephritis and died without receiving therapy for hepatitis C. Case 4 was managed conservatively by decreasing the immunosuppression with regular monitoring.
FCH-C is difficult to treat and shows high morbidity and mortality rates. Treatment is associated with a risk of graft rejection.
纤维淤胆型丙型肝炎(FCH-C)是一种罕见病症,发生于免疫功能低下的患者,是由于高细胞内病毒载量的直接肝毒性以及免疫系统无效,最终导致致命后果。我们在此描述了4例在过去8个月内在我院被诊断为FCH-C的肾移植受者。
4例肾移植受者出现黄疸且肝功能检查结果恶化。根据丙型肝炎病毒(HCV)RNA的存在及特征性肝活检结果,他们被诊断为FCH-C;没有证据表明存在胆汁淤积或胆道梗阻的任何其他原因。
患者为40岁、25岁、20岁和27岁的男性。移植后的时间分别为1.5年、10年、1.5年和2.0年。所有病例移植前HCV抗体、HCV RNA和乙肝表面抗原(HBsAg)筛查均为阴性。所有4例患者均感染1型,而病例2合并感染3型。接受干扰素和利巴韦林治疗的病例1和病例2,胆汁淤积有所改善,但未实现快速病毒学应答。病例1在开始干扰素治疗4个月后因急性细胞排斥反应继发移植功能障碍,接受了脉冲类固醇治疗。由于全血细胞减少,两例均过早停止了基于干扰素的治疗。病例3发生严重肾盂肾炎,未接受丙型肝炎治疗即死亡。病例4通过减少免疫抑制并定期监测进行保守治疗。
FCH-C难以治疗,发病率和死亡率高。治疗伴有移植排斥反应的风险。