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SZ phenotype alpha‐1‐antitrypsin deficiency with paucity of the interlobular bile ducts
Author(s) -
DORNEY S.F. A.,
HASSALL E. G.,
ARBUCKLE S. M.,
VARGAS J. H.,
BERQUIST W. E.
Publication year - 1987
Publication title -
journal of paediatrics and child health
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.631
H-Index - 76
eISSN - 1440-1754
pISSN - 1034-4810
DOI - 10.1111/j.1440-1754.1987.tb02177.x
Subject(s) - medicine , alpha 1 antitrypsin deficiency , phenotype , pathology , liver biopsy , congenital hepatic fibrosis , gastroenterology , biopsy , bile duct , liver disease , portal hypertension , cirrhosis , gene , biology , biochemistry
Abstract A child is reported whose alpha‐1 ‐antitrypsin phenotype is SZ and who has chronic cholestatic liver disease that began in the neonatal period. Liver biopsy demonstrated paucity of the interlobular bile ducts, marked hepatocellular deposition in periportal areas of PAS‐positive, diastase‐resistant granules, and bridging portal fibrosis. The association of paucity of the interlobular bile ducts with SZ phenotype alpha‐1‐antitrypsin deficiency has not been reported previously.