JUVENILE CIRRHOSIS AND MEMBRANOUS GLOMERULONEPHRITIS IN A CHILD WITH ALPHA1ANTITRYPSIN DEFICIENCY PiSZ

ABSTRACT. An infant with alpha₁-antitrypsin (α₁-AT) deficiency PiSZ presented with liver cirrhosis and showed clinical and laboratory evidence of renal disease when hepatic decompensation developed, shortly before death at 12 months of age. Low serum levels of α₁-AT were only demonstrated late in the disease. SZ phenotype was proved by starch gel electrophoresis. Post-mortem pathological studies revealed sevre hepatic cirrhosis with intracytoplasmic inclusion of α₁-AT and membranous glomerulonephritis with deposits of complement and immunoglobulins but without the presence of α₁-AT. The present case suggests the importance of studying Pi phenotypes and serum levels of α₁-AT in all cases of idiopathic cirrhosis or renal disease in infancy.