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Congenital Fibrosis and Polycystic Disease of Liver and Kidneys

M. Michael Thaler, MD; Edward S. Ogata, MD; Joseph R. Goodman, PhD; Carolyn F. Piel, MD; Melvyn T. Korobkin, MD
Am J Dis Child. 1973;126(3):374-380. doi:10.1001/archpedi.1973.02110190330019.
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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


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