Biliary liver cirrhosis secondary to cystic fibrosis: a rare indication for liver transplantation

Transplantation Proceedings
J Sańko-ResmerM Krawczyk

Abstract

As more effective therapies prolong the lives of patients with cystic fibrosis, there are now more patients in this population diagnosed with liver diseases. Secondary biliary cirrhosis is not a rare complication of mucoviscidosis. It is diagnosed in 20% of patients with mucoviscidosis; in 2% it is accompanied by portal hypertension. On average patients with portal hypertension and its complications are 12 years old. Liver transplantation is an accepted method of treatment for children with cystic fibrosis and portal hypertension. It eliminates the cause of the portal hypertension, decreases life-threatening medical conditions, and improves their nutritional status and quality of life. Despite immunosuppressive treatment they do not seem to beat increased risk of upper respiratory tract infections. On the contrary improved respiratory function and status are generally observed. We present our first case of orthotopic liver transplantation performed in a 29-year-old man with cystic fibrosis. The donor was a 42-year-old woman who died of a ruptured cerebral aneurysm. The surgery was performed in September 2004. The patient received immunosuppression based on steroids, basiliximab, tacrolimus, and mycophenolic acid due to renal in...Continue Reading

References

Nov 14, 2001·Current Opinion in Pulmonary Medicine·Y S GenykD W Thomas
Apr 23, 2002·Journal of Gastroenterology and Hepatology·Piotr MilkiewiczElwyn Elias
Sep 25, 2002·Transplantation Proceedings·E PfisterM Melter
Nov 26, 2002·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Carla ColomboAnnamaria Giunta
Dec 8, 2004·Journal of Hepatology·Thierry LamireauFernando Alvarez
Feb 5, 2005·Transplant International : Official Journal of the European Society for Organ Transplantation·Carla ColomboAlberto Battezzati

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