Liver transplantation in cystic fibrosis

Abstract

Liver disease is the second most common cause of death in patients with cystic fibrosis (CF). Improvement in surgical techniques, medical management, and imaging modalities has broadened the range of options for treatment of these patients. Medical management with ursodeoxycholic acid and nutritional support may help decelerate the progression of liver disease. A timely evaluation of CF patients with liver involvement for transplantation is important. Such evaluation should not be delayed until signs of hepatic decompensation occur. Combined lung-liver transplant can be considered for patients with advanced pulmonary disease. Pretransplant management of portal hypertension with a portosystemic shunt procedure is an option for patients with well-preserved synthetic liver function. Improvement in lung function after liver transplantation and no significant risk of pulmonary infection with immunosuppressive therapy have been reported. Review of individual center experiences have shown satisfactory survival and improved quality of life for CF patients undergoing liver transplant.

Hepatocellular carcinoma in association with cirrhosis in a patient with cystic fibrosis

“…Cystic fibrosis liver disease (CFLD) is the second most common cause of death in patients with CF [1]. The pathogenesis is due to the presence of an abnormal cystic fibrosis transmembrane conductance regulator (CFTR) expressed on the biliary epithelium [2].…”

Radiological Features of Cystic Fibrosis

“…Patients are more frequently asymptomatic, but liver disease is the second most common cause of death in CF (2.2% of deaths) and approximately 6-8% of individuals with CF have potentially fatal liver disease that requires liver transplantation (Genyk et al, 2001). It is thought to be a consequence of thickened secretions and abnormal bile flow within the liver causing bile duct obstruction.…”

Lebertransplantation bei metabolischen Lebererkrankungen im Erwachsenenalter

“…Inzwischen erreichen die meisten Patienten das Erwachsenenalter. Dadurch steigt die klinische Bedeutung der Leberbeteiligung, die bereits die zweithäufigste Todesursache darstellt [90].…”

“…Die Mortalität im längerfristigen Verlauf scheint aber signifikant durch die Lungenerkrankung beeinflusst [96,97]. Insgesamt legen die bisherigen Ergebnisse nahe, mit der Diskussion der Lebertransplantation nicht bis zur hepatischen Dekompensation zu warten, sondern diese frühzeitig anzustreben [90]. Dann lassen sich Ergebnisse erzielen, die sowohl bezüglich der Lebensqualität als auch bezüglich der Überlebensrate mit anderen Erkrankungen vergleichbar sind.…”