Neonatal hepatitis and alpha-1-antitrypsin deficiency. The prognosis in five patients.
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.
D000328:Adult; D002648:Child; D006505:Hepatitis / Q000175:diagnosis* / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000175:diagnosis*; D008099:Liver / Q000473:pathology; D008297:Male; D011379:Prognosis; D019896:alpha 1-Antitrypsin Deficiency