Infantile Cirrhosis With Hereditary Alpha1-Antitrypsin Deficiency

Two male siblings had the onset of liver disease in the early months of life; cirrhosis was demonstrated on open liver biopsy in one, and both were found to have hereditary ZZ alpha1-antitrypsin deficiency in a family genetic study. Both infants have shown significant improvement and are clinically well at the ages of 18 months and 3 years, respectively. The infantile cirrhosis associated with severe alpha1-antitrypsin deficiency does not necessarily pursue a continuous, relentless, down-hill course.