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迟发性肝衰竭:临床特征、血清学及移植后的结局

Late-onset hepatic failure: clinical features, serology and outcome following transplantation.

作者信息

Ellis A J, Saleh M, Smith H, Portmann B, Gimson A, Williams R

机构信息

Institute of Liver Studies King's College Hospital, London, U.K.

出版信息

J Hepatol. 1995 Oct;23(4):363-72. doi: 10.1016/0168-8278(95)80193-6.

Abstract

A further series of 41 adult patients with late-onset hepatic failure was investigates with respect to aetiological factors, particularly hepatitis C and E, which have been identified since our earlier report of this condition. The increased use of transplantation and its impact on survival overall is assessed. Comparison is made with 64 patients admitted over the same period with fulminant hepatic failure of non-A, non-B aetiology. Screening for the hepatitis viruses revealed three cases of hepatitis A and one case of Epstein Barr virus hepatitis. There were no cases of hepatitis C or hepatitis E virus detected by enzyme immunoassay and reverse transcriptase/polymerase chain reaction techniques, although three patients had positivity for IgG anti-hepatitis E virus, demonstrating previous exposure. Serum autoantibodies in a titre greater than or equal to 1:40 were present in 29% of samples tested and in three cases, titres of SMA or ANF were greater than 1:320. In a further five cases, a potentially hepatotoxic agent had been given within 3 months of the onset of symptoms, leaving the majority of patients (29) with no identifiable cause for their disease. The frequency of symptoms, however, including nausea, abdominal discomfort with the subsequent development of ascites, encephalopathy and renal impairment suggest a similar disease process in these patients. Analysis of liver biopsy material showed similar patterns on all cases of map-like necrosis with nodular regeneration and without other additional features of aetiological significance. Differences in clinical and histological changes for the non-A, non-B fulminant hepatic failure comparison group reflect the tempo of disease process rather than the nature and cause of the liver damage. The introduction of transplantation has led to a marked improvement in survival (39% overall in the earlier series). In the 21 patients in whom transplantation was carried out, the 1-year actuarial survival is currently 55%. Treatment of late-onset hepatic failure with corticosteroids and the use of Prostaglandin E1 and interferon in individual cases has been disappointing, and the emphasis in management should be placed on teh early referral of such patients to a centre offering transplantation.

摘要

对另外41例迟发性肝衰竭成年患者的病因因素进行了调查,尤其是丙型和戊型肝炎,自我们之前报道这种疾病以来,这两种病因已被确认。评估了肝移植使用增加的情况及其对总体生存率的影响。并与同期收治的64例非甲非乙型暴发性肝衰竭患者进行了比较。对肝炎病毒的筛查发现3例甲型肝炎和1例EB病毒肝炎。尽管有3例患者IgG抗戊型肝炎病毒呈阳性,表明既往有感染史,但采用酶免疫测定和逆转录酶/聚合酶链反应技术未检测到丙型或戊型肝炎病毒感染病例。检测样本中29%的血清自身抗体滴度大于或等于1:40,3例患者平滑肌抗体(SMA)或抗核因子(ANF)滴度大于1:320。另有5例患者在症状出现后3个月内曾使用过潜在肝毒性药物,其余大多数患者(29例)病因不明。然而,这些患者的症状出现频率,包括恶心、腹部不适以及随后出现的腹水、肝性脑病和肾功能损害,提示疾病过程相似。肝活检材料分析显示,所有病例均呈现地图样坏死伴结节状再生的相似模式,且无其他具有病因学意义的额外特征。非甲非乙型暴发性肝衰竭对照组的临床和组织学变化差异反映了疾病进程的速度,而非肝损伤的性质和病因。肝移植的引入使生存率有了显著提高(早期系列研究中总体生存率为39%)。在接受肝移植的21例患者中,目前1年预期生存率为55%。用皮质类固醇治疗迟发性肝衰竭以及个别病例中使用前列腺素E1和干扰素的效果令人失望,管理的重点应是尽早将此类患者转诊至提供肝移植的中心。

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