α1-Antitrypsin deficiency and infantile liver disease

Abstract

Infantile liver disease with deficiency of serum a-1-antitrypsin is illustrated by a description of the clinical, biochemical, and pathological findings in two affected families. The simplicity of the diagnostic tests is emphasized. Review of 61 biopsies of liver from children and adolescents provided a further 3 cases. It is prudent to exclude this metabolic defect in children with a history of 'neonatal hepatitis'.