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.
Talamo RC, Feingold M. Infantile Cirrhosis With Hereditary Alpha1-Antitrypsin Deficiency: Clinical Improvement in Two Siblings. Am J Dis Child. 1973;125(6):845–847. doi:https://doi.org/10.1001/archpedi.1973.04160060051011
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