Hirschberger M, Stickler G B
Mayo Clin Proc. 1977 Apr;52(4):241-5.
Five patients with alpha-1-antitrypsin deficiency (PiZ) are reported. All these patients presented with the neonatal hepatitis syndrome and two fo them had developed cirrhosis at ages 5 and 8 years, respectively. Three patients, ages 1, 9 and 21 years, are asymptomatic. The oldest patient, 21 years of age, has only mild histologic changes in the liver. The prognosis for patients with alpha-1 antitrypsin deficiency (PiZ) presenting with neonatal hepatitis is not necessarily grave, a finding that differs from previous observations.
报告了5例α-1抗胰蛋白酶缺乏症（PiZ）患者。所有这些患者均表现为新生儿肝炎综合征，其中2例分别在5岁和8岁时发展为肝硬化。3例患者，年龄分别为1岁、9岁和21岁，无症状。最年长的患者21岁，肝脏仅有轻度组织学改变。以新生儿肝炎为表现的α-1抗胰蛋白酶缺乏症（PiZ）患者的预后不一定严重，这一发现与以往的观察结果不同。
