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Neonatal hepatitis and alpha-1-antitrypsin deficiency. The prognosis in five patients
Insights
Alpha-1 antitrypsin deficiency (PiZ) in infants presenting with neonatal hepatitis may not lead to severe liver disease. Some patients show mild or no symptoms, challenging previous assumptions about prognosis.
Area of Science:
- Hepatology
- Genetics
- Pediatrics
Background:
- Alpha-1 antitrypsin deficiency (PiZ) is a genetic disorder.
- Neonatal hepatitis is a common presentation in infants with PiZ.
Abstract:
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.
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