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Cirrhosis and other liver disease in cystic fibrosis

2012; Elsevier BV; Volume: 12; Issue: 2 Linguagem: Inglês

10.1016/j.jcf.2012.11.010

1873-5010

Thomas Flass, Michael R. Narkewicz,

Cystic Fibrosis Research Advances

Although some degree of liver involvement in CF is common, cirrhosis and subsequent portal hypertension is a rare but serious complication seen in only 5–10% of CF patients. Efforts are underway to better define and understand the nature of this condition and to identify which patients are at risk for the development of cirrhosis. Early detection has proven to be elusive, with many patients identified after cirrhosis and portal hypertension have already developed. Cirrhosis impacts the nutritional and pulmonary status of affected patients, but pulmonary status remains the primary determinant of outcome. In the rare patient with hepatic decompensation or significant complications that cannot be managed medically, liver transplantation may be an option. Future efforts should focus on developing accurate methods of detecting individuals at risk for the development of cirrhosis before it is present and therapeutic trials of potential interventions to mitigate or prevent/reverse fibrosis prior to development of cirrhosis and portal hypertension.