ABSTRACT
Patients with cystic fibrosis can have hepatic involvement as a consequence of the disease, although only an estimated 5% of patients develop cirrhosis and only 2% go on to overt liver disease. The importance of the liver in the maintenance of homeostasis with regard to the fibrinolytic and coagulation systems is evidenced by the large volume of literature available on the subject. The literature regarding the involvement of the liver in normal and abnormal regulation of plasminogen activators, plasminogen activator inhibitors, fibrinolysis in general is somewhat contradictory, and at times, controversial. The liver responds to a number of acute inflammatory stimuli by altering its pattern of protein synthesis and secretion. Patients receiving estrogen replacement therapy or high-dose estrogen for contraception can experience coagulation abnormalities. The advent of technology to generate transgenic mice has led to the establishment of a number of lines that have been constructed to either express genes from other species or to over- or underexpress murine genes.
