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June 1976, Vol 130, No. 6 >
Article | June 1976

α1-Antitrypsin Deficiency and Liver Disease in Children

Joseph A. Burke, MD; John L. Kiesel, MD; John D. Blair, MD
Am J Dis Child. 1976;130(6):621-629. doi:10.1001/archpedi.1976.02120070047010.
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• This report describes the clinical, biochemical, and hepatic morphologic findings in ten children with severe serum α1-antitrypsin deficiency. Genetic protease inhibitor (Pi) phenotyping, using acid-starch gel and crossed antigen-antibody electrophoresis, demonstrated Pi phenotype ZZ in all our cases. In eight patients, manifestations of liver disease appeared during the first year of life. The case reports show that α1-antitrypsin deficiency should be suspected in any child with neonatal hepatitis, unexplained hepatomegaly or splenomegaly, or cirrhosis. In our report, one infant is normal at age 6 months, and one infant had progressive hepatic damage that culminated in liver failure and death at age 6 months. The variable clinical course and prognosis for infants with severe α1-antitrypsin deficiency is well illustrated by these two infants.

(Am J Dis Child 130:621-629, 1976)

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