α1-Antitrypsin Deficiency in Early Childhood

T Sveger - Pediatrics, 1978 - publications.aap.org
Among 200,000 infants screened for α1-antitrypsin (α1-AT) deficiency, 125 Pi Z, 48 Pi SZ, 1
Pi S-, and 2 Pi Z-children were followed up prospectively. Eleven percent of the Pi Z infants
had neonatal cholestasis, and at 2 years of age three of them had cirrhosis. About 50% of
the asymptomatic Pi Z and Pi Z-subjects occasionally had serum alanine aminotransferase
(ALAT) levels above normal, and in 15% of them the levels were probably permanently
increased during the first two years of life. Two previously healthy Pi Z children had transient …