RISK OF CIRRHOSIS AND PRIMARY LIVER-CANCER IN ALPHA-1-ANTITRYPSIN DEFICIENCY
RISK OF CIRRHOSIS AND PRIMARY LIVER-CANCER IN ALPHA-1-ANTITRYPSIN DEFICIENCY
复制标题
DOI：
10.1056/nejm198603203141202
复制
发表时间：
1986-03-20
影响因子：
158.5
通讯作者：
VELEZ, R
中科院分区：
文献类型：
--
作者：
ERIKSSON, S;CARLSON, J;VELEZ, R
先前的报告表明纯合子α1-抗胰蛋白酶缺乏、肝硬化和原发性肝癌之间存在关联。为了评估这些并发症的风险，我们对 1963 年至 1982 年期间在瑞典马尔默市发现的 17 例 α1-抗胰蛋白酶缺乏症尸检病例进行了回顾性研究。研究期间，对全市死亡患者中的 38,250 名（即 68.2%）进行了尸检。根据人群中的纯合子频率，预计其中 21 人患有 α1-抗胰蛋白酶缺乏症。 20 名患者被诊断出该病，17 名患者（1 名儿童和 16 名成人）进行了尸检。每个尸检病例与从同一尸检登记册中选择的四个对照相匹配，并计算 Mantel-Haenszel 比值比 (ORmh)。结果表明，α1-抗胰蛋白酶缺乏与肝硬化（ORmh = 7.8；95%置信限，2.4至24.7）和原发性肝癌（ORmh = 29；95%置信限，3.5至114.3）之间存在密切关系。当数据根据性别分层时，这些关联仅对男性患者具有统计学意义。我们得出的结论是，患有 α1-抗胰蛋白酶缺乏症的男性患肝硬化和原发性肝癌的风险可能更高。明显的男性优势表明外源因素的累加效应。
Previous reports have suggested an association between homozygous .alpha.1-antitrypsin deficiency, cirrhosis, and primary liver cancer. To assess the risk of these complications we conducted a retrospective study based on 17 autopsied cases of .alpha.1-antitrypsin deficiency identified during the period 1963 to 1982 in the city of Malmo, Sweden. During the study period, autopsies were performed in 38,250, or 68.2 percent, of all patients in the city who died. From the homozygote frequency in the population, 21 of these were expected to have .alpha.1-antitrypsin deficiency. The disease had been diagnosed in 20, and autopsies had been performed in 17 (1 child and 16 adults). Each autopsied case was matched with four controls selected from the same autopsy register, and the Mantel-Haenszel odds ratio (ORmh) was calculated. The results indicated a strong relation between .alpha.1-antitrypsin deficiency and cirrhosis (ORmh = 7.8; 95 percent confidence limits, 2.4 to 24.7) and primary liver cancer (ORmh = 29; 95 percent confidence limits, 3.5 to 114.3). When data were stratified according to sex, these associations were statistically significant only for male patients. We conclude that men with .alpha.1-antitrypsin deficiency may be at higher risk for cirrhosis and primary liver cancer. The apparent male predominance suggests the additive effects of exogenous factors.