Cottrall K, Cook P J, Mowat A P
Postgrad Med J. 1974 Jun;50(584):376-80. doi: 10.1136/pgmj.50.584.376.
A prospective epidemiological study of the Neonatal Hepatitis Syndrome in S.E. England showed Alpha-1-Antitrypsin Deficiency (Pi ZZ) to be present in seven out of fifty-two patients. Data are considered from these seven patients, and from a further six cases from outside this area. The nature of acute illness, pathological changes on early liver biopsy, and short-term prognosis show considerable variability, but in general the hepatitis is more severe in patients with Alpha-1-A.T. deficiency than in those in whom no aetiological factor was found.
一项针对英格兰东南部新生儿肝炎综合征的前瞻性流行病学研究显示,52名患者中有7名存在α-1-抗胰蛋白酶缺乏症(Pi ZZ)。本文分析了这7名患者以及该地区以外另外6例患者的数据。急性疾病的性质、早期肝活检的病理变化以及短期预后显示出相当大的变异性,但总体而言,α-1-抗胰蛋白酶缺乏症患者的肝炎比未发现病因的患者更为严重。