Liver Transplantation for Cirrhosis in Cystic Fibrosis

Overview

Journal Can J Gastroenterol

Specialty Gastroenterology

Date 2006 Jul 22

PMID 16858500

Citations 7

Authors

T Lamireau

S Martin

M Lallier

J E Marcotte

F Alvarez

Affiliations

Soon will be listed here.

Abstract

Background: Liver disease is the third most common cause of death in children with cystic fibrosis (CF). Liver transplantation is an effective treatment in children with hepatic failure.

Aims: The objective of the present study was to review the indications and postoperative course of hepatic transplantation in a cystic fibrosis population.

Patients: Five children with CF, at a mean age of 16.5 years, underwent liver transplantation.

Results: All patients showed cirrhosis, portal hypertension and hepatic failure. The main postoperative complication was ascites refractory to treatment in two patients. No significant deterioration of the pulmonary function was noted. Two patients died, one of Hodgkin lymphoma and the other of progressive pulmonary failure.

Conclusion: Liver transplantation was indicated in children with CF when hepatic failure and/or severe portal hypertension was present with well-preserved pulmonary function.

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References

Fridell J, Bond G, Mazariegos G, Orenstein D, Jain A, Sindhi R . Liver transplantation in children with cystic fibrosis: a long-term longitudinal review of a single center's experience. J Pediatr Surg. 2003; 38(8):1152-6. DOI: 10.1016/s0022-3468(03)00260-4. View

Drage M, Jamieson N, Friend P, Noble-Jamieson G, Levy R, Barnes N . Biliary complications in liver transplantation for cystic fibrosis. Transplant Proc. 1999; 31(6):2411-2. DOI: 10.1016/s0041-1345(99)00407-8. View

Mieles L, Orenstein D, Teperman L, Podesta L, Koneru B, Starzl T . Liver transplantation in cystic fibrosis. Lancet. 1989; 1(8646):1073. DOI: 10.1016/s0140-6736(89)92464-1. View

Scott-Jupp R, Lama M, Tanner M . Prevalence of liver disease in cystic fibrosis. Arch Dis Child. 1991; 66(6):698-701. PMC: 1793169. DOI: 10.1136/adc.66.6.698. View

Sokol R, Johnson K, Karrer F, Narkewicz M, Smith D, Kam I . Improvement of cyclosporin absorption in children after liver transplantation by means of water-soluble vitamin E. Lancet. 1991; 338(8761):212-4. DOI: 10.1016/0140-6736(91)90349-t. View

FitzSimmons S . The changing epidemiology of cystic fibrosis. J Pediatr. 1993; 122(1):1-9. DOI: 10.1016/s0022-3476(05)83478-x. View

FEIGELSON J, Anagnostopoulos C, Poquet M, Pecau Y, Munck A, Navarro J . Liver cirrhosis in cystic fibrosis--therapeutic implications and long term follow up. Arch Dis Child. 1993; 68(5):653-7. PMC: 1029335. DOI: 10.1136/adc.68.5.653. View

Greally P, Hussain M, Vergani D, Price J . Serum interleukin-1 alpha and soluble interleukin-2 receptor concentrations in cystic fibrosis. Arch Dis Child. 1993; 68(6):785-7. PMC: 1029377. DOI: 10.1136/adc.68.6.785. View

Cohn J, Strong T, Picciotto M, Nairn A, Collins F, Fitz J . Localization of the cystic fibrosis transmembrane conductance regulator in human bile duct epithelial cells. Gastroenterology. 1993; 105(6):1857-64. DOI: 10.1016/0016-5085(93)91085-v. View

10.

Noble-Jamieson G, Valente J, Barnes N, Friend P, Jamieson N, Rasmussen A . Liver transplantation for hepatic cirrhosis in cystic fibrosis. Arch Dis Child. 1994; 71(4):349-52. PMC: 1030018. DOI: 10.1136/adc.71.4.349. View

11.

Dousset B, Valverde A, Houssin D, Bernard O, Chapuis Y . [Ascites after liver transplantation in children]. Ann Radiol (Paris). 1994; 37(5):386-90. View

12.

Gane E, Langley P, Williams R . Massive ascitic fluid loss and coagulation disturbances after liver transplantation. Gastroenterology. 1995; 109(5):1631-8. DOI: 10.1016/0016-5085(95)90653-3. View

13.

Mack D, Traystman M, Colombo J, Sammut P, Kaufman S, Vanderhoof J . Clinical denouement and mutation analysis of patients with cystic fibrosis undergoing liver transplantation for biliary cirrhosis. J Pediatr. 1995; 127(6):881-7. DOI: 10.1016/s0022-3476(95)70022-6. View

14.

Pescovitz M, Puente J, Jindal R, Fitzgerald J, Chong S, Milgrom M . Improved absorption of cyclosporine for microemulsion in a pediatric liver transplant recipient with cystic fibrosis. Transplantation. 1996; 61(2):331-3. DOI: 10.1097/00007890-199601270-00030. View

15.

Colombo C, Battezzati P, Podda M, Bettinardi N, Giunta A . Ursodeoxycholic acid for liver disease associated with cystic fibrosis: a double-blind multicenter trial. The Italian Group for the Study of Ursodeoxycholic Acid in Cystic Fibrosis. Hepatology. 1996; 23(6):1484-90. DOI: 10.1002/hep.510230627. View

16.

Noble-Jamieson G, Barnes N . Liver transplantation for cirrhosis in cystic fibrosis. J Pediatr. 1996; 129(2):314. DOI: 10.1016/s0022-3476(96)70263-9. View

17.

Selberg O, Bottcher J, Tusch G, Pichlmayr R, Henkel E, Muller M . Identification of high- and low-risk patients before liver transplantation: a prospective cohort study of nutritional and metabolic parameters in 150 patients. Hepatology. 1997; 25(3):652-7. DOI: 10.1002/hep.510250327. View

18.

Cirera I, Navasa M, Rimola A, Garcia-Pagan J, Grande L, Garcia-Valdecasas J . Ascites after liver transplantation. Liver Transpl. 2000; 6(2):157-62. DOI: 10.1002/lt.500060219. View

19.

Molmenti E, Nagata D, Roden J, Squires R, Molmenti H, Casey D . Pediatric liver transplantation for cystic fibrosis. Transplant Proc. 2001; 33(1-2):1738. DOI: 10.1016/s0041-1345(00)02825-6. View

20.

Milkiewicz P, Skiba G, Kelly D, Weller P, Bonser R, Gur U . Transplantation for cystic fibrosis: outcome following early liver transplantation. J Gastroenterol Hepatol. 2002; 17(2):208-13. DOI: 10.1046/j.1440-1746.2002.02671.x. View