The the portal tracts can often be expanded by fibrosis and contain an inflammatory infiltrate. The ductular proliferation is striking.
Cystic fibrosis (CF) is an autosomal-recessive disorder caused by a mutation of the CFTR (cystic fibrosis transmembrane conductance receptor) gene located on chromosome 7q31. Dysfunction of this protein leads to impaired transport of electrolytes across the cell membrane. Although the lung and pancreas are most severely affected, liver complications can also develop as a result of mucous plugging of the bile ducts, cholestasis and biliary fibrosis.
Microscopically, one sees ductular proliferation with eosinophilic concretions within the dilated ductules. The portal tracts will be expanded by fibrosis, and eventually lead to cirrhosis.
Approximately 10% of CF patients develop cirrhosis by age 25, and up to 10% develop diabetes (Iacobuzio, Cheng).
Mortality is largely due to pulmonary complications.
Cheng L, Bostwick DG, eds. Essentials of Anatomic Pathology. 2nd Ed. Totowa, NJ: Humana Press; 2006: 1400.
Iacobuzio-Donahue CA, Montgomery EA. Gastrointestinal and Liver Pathology: Foundations in Diagnostic Pathology. Philadelphia, PA: Elsevier; 2005: 527-8.