Abstract
Histologic changes in the liver which are diagnostic of cystic fibrosis are not frequently recognized during infancy. In a retrospective study of 47 patients surviving up to 3 months of age, only 5 cases of typical focal biliary cirrhosis were identified. In an additional 11 cases, extra or intra-hepatic ducts were prominent because of focal mucus accumulations, in these cases there were diffuse nonspecific alterations, including clinical jaundice or cholestasis with bile thrombi, apparent prominence of portal triads with fibrosis or inflammation, and minimal bile duct proliferation. Similar nonspecific lesions were present in 14 cases in the absence of excess stainable mucus in bile ducts or liver. In only 17 of the young infants (36%) was the liver entirely normal.
Diagnostic focal biliary cirrhosis was present in S of 32 infants who survived from 3 to 12 months and in 18 of 67 older infants, children, and adolescents, but the diffuse nonspecific changes were absent in the older age groups.
These observations suggest that bile ducts containing mucus alone or in combination with bile may be a possible precussor of focal biliary cirrhosis. The frequency of diffuse changes (cholestasis, bile thrombi, or jaundice) also suggests the possibility of proximal obstruction which is transient.
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Oppenheimer, E., Esterly, J. Hepatic Changes in Young Infants with Cystic Fibrosis. Pediatr Res 8, 384 (1974). https://doi.org/10.1203/00006450-197404000-00263
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DOI: https://doi.org/10.1203/00006450-197404000-00263