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PHILIP ROSENTHAL, MD; WILLIAM M. LIEBMAN, MD; M. MICHAEL THALER, MD

Am J Dis Child. 1979;133(11):1195-1196.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

Liver disease in infants with alpha₁-antitrypsin deficiency was first reported by Freier et al in 1968.¹ This type of infantile liver disease has been observed almost exclusively in association with the ZZ phenotype for the protease inhibitor system.² Alpha₁-antitrypsin deficiency with liver disease has also been described in heterozygous adults with phenotype SZ, but has been rarely implicated as a cause of neonatal hepatitis and subsequent chronic liver disease in childhood.^3.4 In addition, the characteristic hepatocellular PAS-positive granules observed in the liver biopsy specimens of pediatric patients with ZZ phenotype have been infrequently observed in other phenotypes.⁵ We are reporting a case of alpha₁-antitrypsin deficiency in an infant with SZ phenotype associated with severe cholestatic liver disease and parenchymal deposition of PAS- positive material.

Report of a Case.—A male infant weighing 1,600 g was delivered by cesarean section at 31 to . . . [Full Text PDF of this Article]

Author Affiliations
Division of Gastroenterology Department of Pediatrics University of California School of Medicine San Francisco, CA 94142

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