Hodges J R, Millward-Sadler G H, Barbatis C, Wright R
N Engl J Med. 1981 Mar 5;304(10):557-60. doi: 10.1056/NEJM198103053041001.
To determine the prevalence of alpha 1-antitrypsin deficiency in patients with cirrhosis or chronic active hepatitis, we performed a five-year prospective study of liver-biopsy specimens from 1055 adults. Thirty-four patients whose specimens contained hepatocyte inclusions characteristic of the deficiency were phenotyped, and 25 had phenotype MZ (2.4 per cent). The distribution of patients with this phenotype among the 185 patients with cirrhosis diagnosed histologically was three of 84 patients with alcoholic cirrhosis (3.5 per cent), seven of 34 with non-B chronic active hepatitis (20.5 per cent), six of 28 with cryptogenic cirrhosis (21 per cent), and one of 39 with other kinds of cirrhosis (2.6 per cent). The increased prevalence of MZ in patients with cryptogenic cirrhosis and with chronic active hepatitis is highly significant (P < 0.001). Because serum levels of alpha 1-antitrypsin may be unreliable for identification of the subgroup of patients with chronic active hepatitis or cryptogenic cirrhosis, analysis of serum for the MZ phenotype and meticulous examination of biopsy specimens may be necessary.
为了确定肝硬化或慢性活动性肝炎患者中α1 -抗胰蛋白酶缺乏症的患病率，我们对1055名成年人的肝活检标本进行了一项为期五年的前瞻性研究。对34例标本中含有该缺乏症特征性肝细胞内含物的患者进行了表型分析，其中25例为MZ表型（占2.4%）。在经组织学诊断的185例肝硬化患者中，该表型患者的分布情况如下：84例酒精性肝硬化患者中有3例（占3.5%），34例非B型慢性活动性肝炎患者中有7例（占20.5%），28例隐源性肝硬化患者中有6例（占21%），39例其他类型肝硬化患者中有1例（占2.6%）。隐源性肝硬化和慢性活动性肝炎患者中MZ表型患病率的增加具有高度显著性（P < 0.001）。由于血清α1 -抗胰蛋白酶水平可能无法可靠地识别慢性活动性肝炎或隐源性肝硬化患者亚组，因此可能有必要分析血清的MZ表型并仔细检查活检标本。
