Search

Search Results

• Journal

  Archives of Bone and Joint Surgery

  Digital Access

  Provider

  Version

  PubMed Central

  v. 1-, 2013-

  AB&JS

  v. 1-, 2013-
  Get Shareable Link

  View Details
• Article

  Juvenile cirrhosis and membranous glomerulonephritis in a child with alpha1-antitrypsin deficiency PiSZ.

  Rodriguez-Soriano J, Fidalgo I, Camarero C, Vallo A, Oliveros R.

  Acta Paediatr Scand. 1978 Nov;67(6):793-6.

  An infant with alpha1-antitrypsin (alpha1-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 alpha1-AT were only demonstrated late in the disease. SZ phenotype was proved by starch gel electrophoresis. Post-mortem pathological studies revealed severe hepatic cirrhosis with intracytoplasmic inclusion of alpha1-AT and membranous glomerulonephritis with deposits of complement and immunoglobulins but without the presence of alpha1-AT. The present case suggests the importance of studying Pi phenotypes and serum levels of alpha1-AT in all cases of idiopathic cirrhosis or renal disease in infancy.

  Digital Access Access Options
  Get Shareable Link

  PubMed