Neonates with cholestasis may have hepatocellular damage without marked inflammatory cell infiltrates, portal fibrosis with duct proliferation, or ductal hypoplasia. Cirrhosis is a late finding. Prenatal diagnosis of 1 -antitrypsin deficiency comes from detecting the Z allele in cells collected by amniocentesis or chorionic villous biopsy. However, even this is not securely predictive of outcome in terms of emphysema or cirrhosis. No proven medical therapy exists, either to stimulate production or to replace it.
Liver transplantation is the only successful therapeutic management for severe hepatic injury.
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