Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease

Journal of Cystic Fibrosis

Volumn 10, Issue SUPPL. 2, 2011, Pages

  Debray, Dominique ^a   Kelly, Deirdre ^b   Houwen, Roderick ^c   Strandvik, Birgitta ^d   Colombo, Carla ^e  

^a HÔPITAL NECKER ENFANTS MALADES   (France)

^b BIRMINGHAM CHILDREN S HOSPITAL   (United Kingdom)

^c UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

^d KAROLINSKA INSTITUTE   (Sweden)

^e UNIVERSITY OF MILAN   (Italy)

Author keywords

Cystic fibrosis; Esophageal varices; Liver biopsy; Liver disease; Ultrasonography; Ursodeoxycholic acid

Indexed keywords

ALANINE AMINOTRANSFERASE; ALFACALCIDOL; ALPHA TOCOPHEROL; ASPARTATE AMINOTRANSFERASE; BETA ADRENERGIC RECEPTOR BLOCKING AGENT; GAMMA GLUTAMYLTRANSFERASE; HEPATITIS A VACCINE; HEPATITIS B VACCINE; PANCREAS ENZYME; RETINOL; URSODEOXYCHOLIC ACID; VITAMIN K GROUP;

ALANINE AMINOTRANSFERASE BLOOD LEVEL; ANOREXIA; ARTICLE; ASPARTATE AMINOTRANSFERASE BLOOD LEVEL; CALORIC INTAKE; CARBOHYDRATE DIET; COMPUTER ASSISTED TOMOGRAPHY; CYSTIC FIBROSIS; CYSTIC FIBROSIS ASSOCIATED LIVER DISEASE; DISEASE ASSOCIATION; DISEASE COURSE; DOPPLER ECHOGRAPHY; DRUG ABSORPTION; DRUG MEGADOSE; ECHOGRAPHY; ELASTOGRAPHY; ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY; ESOPHAGUS VARICES BLEEDING; FOLLOW UP; GAMMA GLUTAMYL TRANSFERASE BLOOD LEVEL; GASTROINTESTINAL HEMORRHAGE; HEPATITIS A; HEPATITIS B; HEPATOMEGALY; HEPATOPULMONARY SYNDROME; HUMAN; LEUKOPENIA; LIVER BIOPSY; LIVER CIRRHOSIS; LIVER DISEASE; LIVER DYSFUNCTION; LIVER FAILURE; LIVER FUNCTION TEST; LIVER SCINTISCANNING; LIVER TRANSPLANTATION; LUNG FUNCTION; MAGNETIC RESONANCE CHOLANGIOPANCREATOGRAPHY; NOSE FEEDING; NUCLEAR MAGNETIC RESONANCE IMAGING; NUTRITIONAL SUPPORT; PATIENT COMPLIANCE; PATIENT REFERRAL; PHYSICAL EXAMINATION; PORTAL HYPERTENSION; PRACTICE GUIDELINE; PROTEIN DIET; RECTUM HEMORRHAGE; SCLEROTHERAPY; SODIUM RESTRICTION; SPLENECTOMY; SPLENOMEGALY; THROMBOCYTOPENIA; TRANSIENT ELASTOGRAPHY; TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC SHUNT; TREATMENT INDICATION; VITAMIN SUPPLEMENTATION;

BENCHMARKING; CYSTIC FIBROSIS; ESOPHAGEAL AND GASTRIC VARICES; HUMANS; LIVER DISEASES; MEDICINE; PRACTICE GUIDELINES AS TOPIC;

EID: 79958134189     PISSN: 15691993     EISSN: 18735010     Source Type: Journal    
DOI: 10.1016/S1569-1993(11)60006-4     Document Type: Article

References (59)

1. Rowe SM, Miller S, Sorscher EJ Mechanisms of disease, Cystic fibrosis. N Engl J Med 2005, 352:1992-2001.
2. Colombo C, Battezzati PM, Crosignani A, et al. Liver disease in Cystic Fibrosis: a prospective study on incidence, risk factors and outcome. Hepatology 2002, 36:1374-1382.
3. Lindblad A, Glaumann H, Strandvik B Natural history of liver disease in Cystic Fibrosis. Hepatology 1999, 30:1151-1158.
4. Debray D, Lykavieris P, Gauthier F, et al. Outcome of cystic fibrosis-associated liver cirrhosis: management of portal hypertension. J Hepatol 1999, 31:77-83.
5. Gooding I, Dondos V, Gyi KM, et al. Variceal hemorrhage and cystic fibrosis: outcomes and implications for liver transplantation. Liver Transpl 2005, 12:1522-1526.
6. Cystic Fibrosis Foundation Patient Registry 2003 Annual Report to the Center Directors 2004, Cystic Fibrosis Foundation, Bethesda, MD.
7. Colombo C, Battezzati PM Liver involvement in Cystic Fibrosis: primary organ damage or innocent bystander?. J Hepatol 2004, 41:1041-1044.
8. Kelly D, Disease of the Liver and Biliary System in Children, Third edition, 2008.
9. Sokol RJ, Durie PR Recommendations for management of liver and biliary tract disease in cystic fibrosis. Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group. J Pediatr Gastroenterol Nutr 1999, 28(Suppl 1):1-13.
10. Bartlett JR, Friedman KJ, Ling SC, et al. Genetic modifiers of liver disease in cystic fibrosis. JAMA 2009, 302:1076-1083.
11. Chen AH, Innis SM, Davidson GF, Jill James S Phosphatidyl-choline and lysophosphatidyl-choline excretion is increased in children with cystic fibrosis and is associated with plasma homocysteine, S-adenosylhomocysteine, and S-adenosylmethionine. Am J Clin Nutr 2005, 81:686-691.
12. Pinto HC, Carniero de Moura M, Day CP Non-alcoholic steatohepatitis from cell biology to clinical practice. J Hepatology 2006, 44:197-208.
13. Strandvik B, Hjelte L, Gabrielsson N, Glaumann H Sclerosing cholangitis in cystic fibrosis. Scand J Gastroenterol Suppl 1988, 143:121-124.
14. Nagel RA, Westaby D, Javaid A, et al. Liver disease and bile duct abnormalities in adults with cystic fibrosis. Lancet 1989, 2:1422-1425.
15. Durieu I, Pellet O, Simonot L, et al. Sclerosing cholangitis in adults with cystic fibrosis: a magnetic resonance cholangiographic prospective study. J Hepatol 1999, 30:1052-1056.
16. Lindblad A, Hultcrantz R, Strandvik B Bile-duct destruction and collagen deposition: a prominent ultrastructural feature of the liver in cystic fibrosis. Hepatology 1992, 16:372-381.
17. Colombo C, Crosignani A, Assaisso ML, et al. Ursodeoxycholic acid therapy in Cystic Fibrosis associated liver disease: a dose-response study. Hepatology 1992, 16:924-930.
18. Colombo C, Castellani MR, Balistreri WF, Seregni E, Assaisso ML, Giunta A Scintigraphic documentation of an improvement in hepatobiliary excretory function after treatment with ursodeoxycholic acid in patients with Cystic Fibrosis and associated liver disease. Hepatology 1992, 15:677-684.
19. Colombo C, Battezzati PM, Podda M, Bettinardi, Giunta A., the Italian Group for the Study of Ursodeoxycholic acid in Cystic Fibrosis Ursodeoxycholic acid for liver disease associated with Cystic Fibrosis: a double-blind multicenter trial. Hepatology 1996, 23:1484-1490.
20. Lindblad A, Glaumann H, Strandvik B A two-year prospective study of the effect of ursodeoxycholic acid on urinary bile acid excretion and liver morphology in Cystic Fibrosis. Hepatology 1998, 23:166-174.
21. Nousia-Arvanitakis S, Futoulaki M, Economou H, et al. Long-term prospective study of the effect of ursodeoxycholic acid on cystic fibrosis-related liver disease. J Clin Gastroenterol 2001, 32:324-328.
22. Wilschanski M, Rivlin J, Cohen S, et al. Clinical and genetic risk factors for CF-related liver disease. Pediatrics 1999, 103:52-57.
23. Lamireau T, Monnereau S, Martin S, et al. Epidemiology of liver disease in cystic fibrosis: a longitudinal study. J Hepatol 2004, 41:920-925.
24. Corbett K, Kelleher S, Rowland M, et al. Cystic fibrosis-associated liver disease: a population-based study. J Pediatr 2004, 145:327-332.
25. Potter CJ, Fishbein M, Hammond S, et al. Can the histologie changes of Cystic Fibrosis-associated hepatobiliary disease be predicted by clinical criteria?. J Pediatr Gastroenterol Nutr 1997, 25:32-36.
26. Shapira R, Hadzic R, Francavilla R, Koukulis G, Price GF, Mieli-Vergani G Retrospective review of cystic fibrosis presenting as infantile liver disease. Arch Dis Child 1999, 81:125-128.
27. Pereira TN, Lewindon PJ, Smith JL, et al. Serum markers of hepatic fibrogenesis in cystic fibrosis liver disease. J Hepatol 2004, 41:576-583.
28. Patriquin H, Lenaerts C, Smith L, et al. Liver disease in children with Cystic Fibrosis: US and biochemical comparison in 195 patients. Radiology 1999, 211:229-232.
29. Lenaerts C, Lapierre C, Patriquin H, et al. Surveillance for cystic fibrosis-associated hepatobiliary disease: early ultrasound changes and predisposing factors. J Pediatr 2003, 143:343-350.
30. Williams SG, Evanson JE, Barrett N, Hodson ME, Boultbee JE, Westaby D An ultrasound scoring system for the diagnosis of liver disease in cystic fibrosis. J Hepatol 1995, 22:513-521.
31. Mueller-Abt PR, Frawley KJ, Gréer RM, Lewindon PJ Comparison of ultrasound and biopsy findings in children with cystic fibrosis related liver disease. J Cyst Fibros 2008, 7:215-221.
32. de Lédinghen V, Le Bail B, Rebouissoux L, et al. Liver stiffness measurement in children using FibroScan: feasibility study and comparison with Fibrotest, aspartate transaminase to platelets ratio index, and liver biopsy. J Pediatr Gastroenterol Nutr 2007, 45:443-450.
33. Foucher J, Chanteloup E, Vergniol J, et al. Diagnosis of cirrhosis by transient elastography (FibroScan): a prospective study. Gut 2006, 55:403-408.
34. Witters P, De Boeck K, Dupont L, et al. Non-invasive liver elastography (Fibroscan) for detection of cystic fibrosis-associated liver disease. J Cyst Fibros 2009, 8:392-399.
35. O'Connor PJ, Southern KW, Bowler IM The role of hepatobiliary scintigraphy in Cystic Fibrosis. Hepatology 1996, 23:281-287.
36. Lawson EE, Grand RJ, Neff RK, Cohen LF Clinical estimation of liver span in infants and children. Am J Dis Child 1978, 132:474-476.
37. Rosenberg HK, Markowitz RI, Kolberg H, Park C, Hubbard A, Bellah RD Normal splenic size in infants and children: sonographic measurements. AJR 1991, 157:119-121.
38. Cheng K, Ashby D, Smyth R. Ursodeoxycholic acid for Cystic Fibrosis-related liver disease (Cochrane Review). In: The Cochrane Library, Issue 2. Oxford: Update Software 6-12, 2000.
39. Colombo C, Roda A, Roda E, et al. Evaluation of an ursodeoxycholic acid oral load in the assessment of bile acid malabsorption in Cystic Fibrosis. Dig Dis Sci 1983, 28:306-311.
40. Rodríguez-Roisin R, Krowka MJ Hepatopulmonary syndrome: a liver-induced lung vascular disorder. N Engl J Med 2008, 358:2378-2387.
41. Herve P, Le Pavec J, Sztrymf B, Decante B, Savale L, Sitbon O Pulmonary vascular abnormalities in cirrhosis. Best Pract Res Clin Gastroenterol 2007, 21:141-159.
42. McKeon D, Day A, Parmar J, Alexander G, Bilton D Hepatocellular carcinoma in association with cirrhosis in a patient with Cystic Fibrosis. J Cyst Fibros 2004, 3:193-195.
43. Kelleher T, Staunton M, O'Mahony S, McCormick PA Advanced hepatocellular carcinoma associated with Cystic Fibrosis. Eur J Gastroenterol Hepatol 2005, 17:1123-1124.
44. de Franchis R Evolving consensus in portal hypertension. Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension. J Hepatol 2005, 43:167-176.
45. Khuroo MS, Khuroo NS, Farahat KL, Khuroo YS, Sofi AA, Dahab ST Meta-analysis: endoscopie variceal ligation for primary prophylaxis of oesophageal variceal bleeding. Aliment Pharmacol Ther 2005, 21:347-361.
46. Pozler O, Krajina A, Vanicek H, et al. Transjugular intrahepatic portosystemic shunt in five children with cystic fibrosis: long-term results. Hepatogastroenterology 2003, 50:1111-1114.
47. Efrati O, Barak A, Modan-Moses D, et al. Liver cirrhosis and portal hypertension in Cystic Fibrosis. Eur J Gastroenterol Hepatol 2003, 15:1073-1078.
48. Louis D, Duc ML, Reix P, et al. Partial splenectomy for portal hypertension in cystic fibrosis related liver disease. Pediatr Pulmonol 2007, 42:1173-1180.
49. Linnane B, Oliver MR, Robinson PJ Does splenectomy in cystic fibrosis related liver disease improve lung function and nutritional status? A case series. Arch Dis Child 2006, 91:771-773.
50. Robberecht E, Van Biervliet S, Vanrentergem K, Kerremans I Outcome of total splenectomy with portosystemic shunt for massive splenomegaly and variceal bleeding in cystic fibrosis. J Pediatr Surg 2006, 41:1561-1565.
51. Minicucci L, Lorini R, Giannattasio A, et al. Liver disease as risk factor for cystic fibrosis-related diabetes development. Acta Paediatr 2007, 96:736-739.
52. Noble-Jamieson G, Barnes N, Jamieson N, Friend P, Cain R Liver transplantation for hepatic cirrhosis in cystic fibrosis. J Royal Soc Med 1996, 89:31-37.
53. Milkiewicz P, Skiba G, Kelly D, et al. Transplantation for cystic fibrosis: outcome following early liver transplantation. J Gastroenterol Hepatol 2002, 17:208.
54. Couetil JP, Soubrane O, Houssin DP, et al. Combined heart-lung-liver, double lung-liver and isolated liver transplantation for cystic fibrosis in children. Transpl Int 1997, 10:33-39.
55. Fridell JA, Bond GJ, Mazariegos GV, et al. Liver transplantation in children with cystic fibrosis: a long-term longitudinal review of a single center's experience. J Pediatr Surg 2003, 38:1152-1156.
56. Melzi ML, Kelly D, Colombo C, et al. Liver transplant in Cystic Fibrosis: a poll among European centers. A study from the European Liver Transplant Registry. Transpl Int 2006, 19:726-731.
57. Nash KL, Collier JD, French J, et al. Cystic fibrosis liver disease: to transplant or not to transplant?. Am J Transplant 2008, 8:162-169.
58. Colombo C, Costantini D, Rocchi A, et al. Effects of liver transplantation on the nutritional status of patients with Cystic Fibrosis. Transpl Int 2005, 18:246-255.
59. Moyer K, Balistreri W Hepatobiliary disease in patients with cystic fibrosis. Curr Opin Gastroenterol 2009, 25:272-278.