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JUVENILE CIRRHOSIS AND MEMBRANOUS GLOMERULONEPHRITIS IN A CHILD WITH ALPHA 1 ANTITRYPSIN DEFICIENCY PiSZ
Author(s) -
RODRIGUEZSORIANO J.,
FIDALGO I.,
CAMARERO C.,
VALLO A.,
OLIVEROS R.
Publication year - 1978
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1978.tb16263.x
Subject(s) - medicine , cirrhosis , glomerulonephritis , decompensation , pathology , antibody , liver disease , alpha 1 antitrypsin deficiency , immunology , gastroenterology , kidney
. An infant with alpha 1 ‐antitrypsin (α 1 ‐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 α 1 ‐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 α 1 ‐AT and membranous glomerulonephritis with deposits of complement and immunoglobulins but without the presence of α 1 ‐AT. The present case suggests the importance of studying Pi phenotypes and serum levels of α 1 ‐AT in all cases of idiopathic cirrhosis or renal disease in infancy.