Screening for hepatocellular carcinoma in acute intermittent porphyria: a 15-year follow-up in northern Sweden
Screening for hepatocellular carcinoma in acute intermittent porphyria: a 15-year follow-up in northern Sweden
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DOI：
10.1111/j.1365-2796.2010.02335.x
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发表时间：
2011-05-01
影响因子：
11.1
通讯作者：
Andersson, C.
中科院分区：
文献类型：
--
作者：
Innala, E.;Andersson, C.
Innala E，Andersson C(Umea大学，Umea，瑞典)。急性间歇性门静脉症患者的肝细胞癌筛查：瑞典北部15年的随访研究。目的：评价急性间歇性卟啉(AIP)基因携带者筛查肝细胞癌(HCC)的效益，并估算该人群每年的肝癌发病率。研究对象：所有55岁的AIP基因携带者在1994-2009年间每隔1-1.5年进行一次前瞻性研究。我们在瑞典北部地区(四个县)的AIP基因携带者中登记了所有的肝癌病例。我们比较了重复筛查间隔两年的基因携带者(A组)和对照组(B组，即从未筛查过的基因携带者，第一次筛查或每隔两年筛查的基因携带者，或辍学)。结果：共有62名AIP受试者参与了这项研究，占瑞典北部地区55岁AIP总人口的33%。22名AIP受试者(12名男性和10名女性)被诊断为肝细胞癌，平均年龄69岁(59-82岁)。在这些受试者中，73%的人有过AIP发作的经历。肝癌发病率比为(男性52例，女性93例)。未发现乙型/丙型肝炎或酗酒病例。肝硬变是罕见的。与未定期筛查的患者相比，筛查可提高患者的3年和5年生存率(P=0.005和0.038)。结论：筛查AIP携带者的肝细胞癌可以早期诊断和选择潜在的治疗方法，改善预后。我们建议对50岁的AIP基因携带者每年进行一次肝脏成像筛查。
Innala E, Andersson C (Umea University, Umea, Sweden). Screening for hepatocellular carcinoma in acute intermittent porphyria: a 15-year follow-up in northern Sweden. J Intern Med 2011; 269: 538-545.Objectives.To evaluate the benefit of screening for hepatocellular carcinoma (HCC) in gene carriers of acute intermittent porphyria (AIP) and estimate the annual incidence of HCC in this group.Subjects.All AIP gene carriers aged >= 55 years from the northernmost county in Sweden, Norrbotten, were invited for screening in this prospective study every 1-1.5 years during the period 1994-2009. We registered all HCC cases amongst AIP gene carriers in the northern region of Sweden (four counties). We compared gene carriers with repeated screening intervals of < 2 years (Group A) with controls (Group B; i.e. gene carriers who had never been screened, those screened for the first time or screened at intervals of > 2 years, or dropouts). The screening included radiological examination of the liver and relevant laboratory tests.Results.A total of 62 AIP subjects participated in the study, comprising 33% of the total AIP population aged > 55 years in the northern region of Sweden. HCC was diagnosed in 22 AIP subjects (12 men and 10 women), mean age 69 (59-82) years. Amongst these subjects, 73% had experienced prior AIP attacks. The incidence rate ratio for HCC was 64 (52 in men and 93 in women). There were no cases of hepatitis B/C or alcohol abuse. Liver cirrhosis was rare. Liver resection could be performed in most subjects in Group A. Fourteen patients died of HCC, one in Group A and 13 in Group B. Compared with those who were not screened regularly, screening was associated with improved 3-year and 5-year survival (P = 0.005 and 0.038).Conclusions.Screening for HCC in carriers of AIP enables early diagnosis and a choice of potentially curative treatments with improved prognosis. We recommend annual screening using liver imaging for AIP gene carriers > 50 years of age.