September 1973

Congenital Fibrosis and Polycystic Disease of Liver and Kidneys

Author Affiliations

San Francisco
From the departments of pediatrics (Drs. Thaler, Ogata, and Piel) and radiology (Dr. Korobin), University of California and Veterans Administration Hospital (Dr. Goodman), San Francisco.

Am J Dis Child. 1973;126(3):374-380. doi:10.1001/archpedi.1973.02110190330019

Roentgenographic, surgical, and histologic investigations demonstrated the presence of macrocysts and microcysts in kidneys and liver of a 15-year-old girl with systemic and portal hypertension, thrombocytopenia, and hepatosplenomegaly. Ultrastructural studies showed extensive deposits of collagen interposed between hepatocytes and proliferating biliary structures and distributed around dilated renal tubules. The results of these studies suggest that excessive and abnormally distributed connective tissue may represent the underlying defect in polycystic disease of the liver and kidneys and in congenital hepatic fibro