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Intervenciones para la prevención y el tratamiento de la enfermedad hepática avanzada en la fibrosis quística

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Referencias

References to studies excluded from this review

Ir a:

Belli 1987 {published data only}

Belli DC, Levy E, Darling P, Leroy C, Lepage G, Giguere R, et al. Taurine improves the absorption of a fat meal in patients with cystic fibrosis. Pediatrics 1987;80(4):517‐23. [CENTRAL: 50249; CFGD Register: CO1; CRS: 5500100000000311; PUBMED: 3658570]CENTRAL

Bittner 1991 {published data only}

Bittner P, Posselt HG, Sailer T, Ott H, Magdorf K, Wahn U, et al. The effect of treatment with ursodeoxycholic acid in cystic fibrosis and hepatopathy: results of a placebo‐controlled study. Bile Acids As Therapeutic Agents. From Basic Science to Clinical Practice. Falk Symposium 58. 1991;Falk Symposium 58:345‐8. [CENTRAL: 220490; CFGD Register: CO7b; CRS: 5500100000001144]CENTRAL
Bittner P, Seiler T, Ott H, Posselt H‐G, Margdorf K, Kawinkel M. Therapeutical approach of ursodeoxycholic acid in cystic fibrosis and hepatopathy. 16th Annual Meeting of the European Working Group for Cystic Fibrosis; 1989; Prague, Czechoslovakia. 1989:72. [CENTRAL: 291213; CFGD Register: CO7a; CRS: 5500100000001287]CENTRAL

Colombo 1992 {published data only}

Colombo C, Crosignani A, Assaisso M, Battezzati PM, Podda M, Giunta A, et al. Ursodeoxycholic acid therapy in cystic fibrosis‐associated liver disease: a dose‐response study. Hepatology (Baltimore, Md.) 1992;16(4):924‐30. [CENTRAL: 208420; CFGD Register: CO4b; CRS: 5500100000001071; PUBMED: 1398498]CENTRAL
Colombo C, Setchell KDR, Podda M, Crossignani A, Assaisso ML, Giunta A. Ursodeoxycholic Acid (UDCA) in CF associated liver disease (LD): A dose response study. 17th European Cystic Fibrosis Conference; 1991 Jun 18‐21; Copenhagen, Denmark. 1991:140. [CENTRAL: 291253; CFGD Register: CO4a; CRS: 5500100000001318]CENTRAL

Colombo 1996 {published data only}

Colombo C, Allocca M, Quattrucci S, Traverso G, Farina S, Lucidi V, et al. Ursodeoxycholic acid for liver disease associated to cystic fibrosis: long‐term follow‐up of patients enrolled in the Italian multicenter trial. Pediatric Pulmonology 2005;40(Suppl 28):343. [CENTRAL: 593027; CFGD Register: CO8e; CRS: 5500100000002988]CENTRAL
Colombo C, Battezzati PM, 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 (Baltimore, Md.) 1996;23(6):1484‐90. [CENTRAL: 125471; CFGD Register: CO8c; CRS: 5500100000000751; PUBMED: 8675168]CENTRAL
Colombo C, Battezzati PM, Santini B, Iapichino L, Quattrucci S, Lucidi V, et al. Treatment with Ursodeoxycholic acid for patients with cystic fibrosis and liver disease. Clinical Ecology of Cystic Fibrosis 1993;18:275‐9. [CENTRAL: 444883; CFGD Register: CO8d; CRS: 5500100000002341]CENTRAL
Colombo C, Battezzati PM, Santini B, Lapichino L, Quattrucci S, Lucidi V, et al. Ursodeoxycholic Acid (UDCA) for liver disease associated with cystic fibrosis (CF): a double‐blind multicenter trial. 18th European Cystic Fibrosis Conference; 1993 May 21‐26; Madrid, Spain. 1993:W6.1. [CENTRAL: 291252; CFGD Register: CO8a; CRS: 5500100000001317]CENTRAL
Colombo C, Podda M, Battezzati PM, Santini B, Iapichino L, Quattrucci S, et al. Ursodeoxycholic acid for cystic fibrosis associated liver disease: final report of a multicenter trial. Hepatology (Baltimore, Md.) 1993;18:142A. [CENTRAL: 208167; CFGD Register: CO8b; CRS: 5500100000001048]CENTRAL

Colombo 2002 {published data only}

Colombo C, Battezzati PM, Crosignani A. Liver disease in cystic fibrosis: a prospective study on incidence, risk factors and outcome. Hepatology 2002;36:1374‐82. CENTRAL

Darling 1985 {published data only}

Darling PB, Lepage G, Leroy C, Masson P, Roy C. Effect of taurine supplements on fat absorption in cystic fibrosis. Pediatric Research 1985;19(6):578‐82. [CENTRAL: 38640; CFGD Register: CO12; CRS: 5500100000000231; PUBMED: 4011338]CENTRAL

Fleet 2000 {published data only}

Fleet M, Stanley AJ, Forrest EH, Hayes PC, Redhead DN. Transjugular intrahepatic portosystemic stent shunt placement in a patient with cystic fibrosis complicated by portal hypertension. Clinical Radiology 2000 May;55(3):236‐7. CENTRAL

Kapustina 2000 {published data only}

Kapustina TJ, Kashirskaia NJ, Kapranov NI, Neudakhin EV. Effect of ursodeoxycholic acid on lipid metabolism in patients with cystic fibrosis. 13th International Cystic Fibrosis Congress; 2000 Jun 4‐8; Stockholm, Sweden. 2000:132. [CENTRAL: 302959; CFGD Register: CO14; CRS: 5500100000001693]CENTRAL

Lamireau 2006 {published data only}

Lamireau T, Martin S, Lallier M, Marcotte JE, Alvarez F. Liver transplantation for cirrhosis in cystic fibrosis. Canadian Journal of Gastroenterology 2006;20(7):475‐8. CENTRAL

Lepage 1997 {published data only}

Lacaille F, Paradis K, Lenaerts C, Senechal L, Lepage G, Roy CC, et al. Ursodeoxycholic acid (UDCA) improves essential fatty acid (EFA) deficiency in cystic fibrosis. Hepatology (Baltimore, Md.) 1993;18(4 Pt 2):271A. [CENTRAL: 221437; CFGD Register: CO13b; CRS: 5500100000001145]CENTRAL
Lepage G, Paradis K, Lacaille F, Senechal L, Ronco N, Champagne J, et al. Ursodeoxycholic acid improves the hepatic metabolism of essential fatty acids and retinol in children with cystic fibrosis. Journal of Pediatrics 1997;130(1):52‐8. [CENTRAL: 135934; CFGD Register: CO13a; CRS: 5500100000000805; PUBMED: 9003851]CENTRAL

Merli 1994 {published data only}

Merli M, Bertasi S, Servi R, Diamanti S, Martino F, De Santis A, et al. Effect of a medium dose of ursodeoxycholic acid with or without taurine supplementation on the nutritional status of patients with cystic fibrosis: a randomised, placebo‐controlled, crossover trial. Journal of Pediatric Gastroenterology and Nutrition 1994;19(2):198‐203. [CENTRAL: 108869; CFGD Register: CO5; CRS: 5500100000000659; EMBASE: 1994299511; PUBMED: 7815243]CENTRAL

Mieles 1991 {published data only}

Mieles LA, Orenstein DM, Toussaint RM, Selby R, Gordon RD, Starzl TE. Outcome after liver transplantation for cystic fibrosis. Pediatric Pulmonology. Supplement 1991;11(S1):130‐1. CENTRAL

Narkewicz 1994 {published data only}

Narkewicz MR, Sokol RJ, Lear JL, Wagener JS, Accurso FJ. Effect of ursodeoxycholic acid (UDCA) therapy for CF liver disease on indirect tests of hepatic function. Pediatric Pulmonology 1994;Suppl 10:342. [CENTRAL: 291473; CFGD Register: CO10; CRS: 5500100000001500]CENTRAL

Nightingale 2010 {published data only}

Nightingale S, O'Loughlin EV, Dorney SF, Shun A, Verran DJ, Strasser SI, et al. Isolated liver transplantation in children with cystic fibrosis‐‐an Australian experience. Pediatric Transplantation 2010;14(6):779‐85. CENTRAL

Noble‐Jamieson 1994 {published data only}

Noble‐Jamieson G, Valente J, Barnes ND, Friend PJ, Jamieson NV, Rasmussen A, et al. Liver transplantation for hepatic cirrhosis in cystic fibrosis. Archives of Disease in Childhood 1994;71(4):349‐52. CENTRAL

O'Brien 1992 {published data only}

O'Brien S, Fitzgerald MX, Hegarty JE. A controlled trial of ursodeoxycholic acid treatment in cystic fibrosis‐related liver disease. European Journal of Gastroenterology & Hepatology 1992;4(10):857‐63. [CENTRAL: 196428; CFGD Register: CO11a; CRS: 5500100000001004; EMBASE: 1992292460]CENTRAL
O'Brien S, Fitzgerald MX, Hegarty JE. Ursodeoxycholic acid treatment in cystic fibrosis related liver disease. Gut 1992;33:S14. [CENTRAL: 208507; CFGD Register: CO11b; CRS: 5500100000001089]CENTRAL
O'Brien SM, Campbell GR, Burke AF, Maguire OC, Rowlands BJ, FitzGerald MX, et al. Serum bile acids and ursodeoxycholic acid treatment in cystic fibrosis‐related liver disease. European Journal of Gastroenterology & Hepatology 1996;8(5):477‐83. [CENTRAL: 129951; CFGD Register: CO11c; CRS: 5500100000000779; PUBMED: 8804877]CENTRAL

Pozler 2003 {published data only}

Pozler O, Krajina A, Vanicek H. Transjugular intrahepatic portosystemic shunt in five children with cystic fibrosis: long‐term results. Hepatogastroenterology 2003;50:1111‐4. CENTRAL

Pukhalsky 2008 {published data only}

Pukhalsky A, Shmarina G, Pukhalskaya D, Perederko L. Whether low frequency of hepatobiliary abnormalities in cystic fibrosis patients is associated with anti‐inflammatory treatment?. European Respiratory Society Annual Congress; 2008 Oct 4‐8; Berlin, Germany.. 2008:216s. [CENTRAL: 758640; CFGD Register: IB93; CRS: 5500100000003483]CENTRAL

Roland 2011 {published data only}

Rowland M, Gallagher CG, Laoide RO, Canny G, Broderick A, Hayes R, et al. Outcome in cystic fibrosis liver disease. American Journal of Gastroenterology 2011;106:104‐9. CENTRAL

Schuster 1977 {published data only}

Schuster SR, Shwachman H, Toyam WM, Rubino A, Taik‐Khaw K. The management of portal hypertension in cystic fibrosis. Journal of Pediatric Surgery 1977;12(2):201‐6. CENTRAL

Smith 1991 {published data only}

Smith LJ, Lacaille F, Lepage G, Ronco N, Lamarre A, Roy CC. Taurine decreases fecal fatty acid and sterol excretion in cystic fibrosis. A randomized double‐blind trial. American Journal of Diseases of Children 1991;145(12):1401‐4. [CENTRAL: 98496; CFGD Register: CO3; CRS: 5500100000000606; PUBMED: 1669669]CENTRAL

Spray 1998 {published data only}

Spray C, Sinha B, Raman M, Ramani P, Weller P, Kelly D. Does ursodeoxycholic acid improve histological changes in liver disease in cystic fibrosis?. Journal of Pediatric Gastroenterology and Nutrition 1998;26(5):584. [CENTRAL: 543167; CFGD Register: CO6b; CRS: 5500050000000030]CENTRAL
Spray C, Sinha B, Venkataraman M, Davies P, Ramani P, Weller P, et al. The role of ursodeoxycholic acid on histological changes in children with cystic fibrosis‐liver disease ‐ a prospective study. 13th International Cystic Fibrosis Congress; 2000 Jun 4‐8; Stockholm, Sweden. 2000:132. [CENTRAL: 302984; CFGD Register: CO6a; CRS: 5500100000001713]CENTRAL

Thomas 1995 {published data only}

Thomas PS, Bellamy M, Geddes D. Malabsorption of vitamin E in cystic fibrosis improved after ursodeoxycholic acid. Lancet 1995;346(8984):1230‐1. [CENTRAL: 119931; CFGD Register: GN77; CRS: 5500100000000723; PUBMED: 7475686]CENTRAL

Thompson 1987 {published data only}

Thompson GN, Robb TA, Davidson GP. Taurine supplementation, fat absorption, and growth in cystic fibrosis. Journal of Pediatrics 1987;111(4):501‐6. [CENTRAL: 50200; CFGD Register: CO2a; CRS: 5500100000000309; EMBASE: 1987208025; PUBMED: 3309233]CENTRAL
Thompson GN, Tomas FM. Protein metabolism in cystic fibrosis: responses to malnutrition and taurine supplementation. American Journal of Clinical Nutrition 1987;46(4):606‐13. [CENTRAL: 50330; CFGD Register: CO2b; CRS: 5500100000000313; EMBASE: 1988005723; PUBMED: 3661477]CENTRAL

Van de Meeberg 1997 {published data only}

Van de Meeberg PC, VanBerge Henegouwen GP, The DCF‐UDCATG. Long‐term follow‐up of low dose versus high dose ursodeoxycholic acid (UDCA) in cholestasis related to cystic fibrosis (CF). Gut 1996;39(Suppl 3):A118. [CENTRAL: 208505; CFGD Register: CO9b; CRS: 5500100000001087]CENTRAL
van de Meeberg PC, Houwen RH, Sinaasappel M, Heijerman HG, Bijleveld CM, Vanberge Henegouwen GP. Low‐dose versus high‐dose ursodeoxycholic acid in cystic fibrosis‐related cholestatic liver disease. Results of a randomised study with 1‐ year follow‐up. Scandinavian Journal of Gastroenterolology 1997;32(4):369‐73. [CENTRAL: 139270; CFGD Register: CO9a; CRS: 5500100000000821; PUBMED: 9140160]CENTRAL

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Characteristics of studies

Characteristics of excluded studies [ordered by study ID]

Study

Reason for exclusion

Belli 1987

Examined the effects of taurine supplementation on the absorption of a fat meal.

Bittner 1991

Evaluated the effect of UDCA in CF and hepatopathy. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.

Colombo 2002

Not a RCT. Prospective observational study on incidence, risk factors and outcome of CFLD.

Colombo 1992

Evaluated the effect of UDCA in CF liver disease. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.

Colombo 1996

Long‐term follow‐up study evaluating UDCA for CFLD. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.

Darling 1985

Examined the effects of taurine supplementation on fat absorption in CF.

Fleet 2000

Case study, not a RCT.

Kapustina 2000

Evaluated the effect of UDCA on lipid metabolism in people with CF. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.

Lamireau 2006

Not a RCT.

Lepage 1997

Examined the effect of UDCA on the hepatic metabolism of essential fatty acid in children with CF. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.

Merli 1994

Examined the effect of a medium dose of UDCA with or without taurine supplementation on the nutritional status of people with CF. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.

Mieles 1991

Not a RCT.

Narkewicz 1994

Evaluated the effect of UDCA for CFLD on indirect tests of hepatic function. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.

Nightingale 2010

A retrospective non‐RCT.

Noble‐Jamieson 1994

Non‐randomised study.

O'Brien 1992

Assessed the effect of UDCA in CFLD. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.

Pozler 2003

Not a RCT.

Pukhalsky 2008

Evaluated anti‐inflammatory treatment on low frequency hepato‐biliary abnormalities in people with CF.

Roland 2011

Not a RCT, a cohort study.

Schuster 1977

Not a RCT, a cohort study.

Smith 1991

Examined the effects of taurine on faecal fatty acid and sterol excretion in CF.

Spray 1998

Prospective study of the role of UDCA on histological changes in children with CFLD. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.

Thomas 1995

Examined the effect of UDCA on malabsorption of vitamin E in CF. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.

Thompson 1987

Assessed protein metabolism in CF, response to malnutrition and taurine supplementation.

Van de Meeberg 1997

Long‐term follow up of low‐dose versus high‐dose UDCA in cholestasis related to CF. Treatment with UDCA is not a relevant treatment to this review as its main purpose of use is to delay the progression of CFLD. Our review evaluates the interventions for advanced liver disease in cystic fibrosis; i.e. interventions for prevention (primary and secondary) and treatment of oesophageal variceal bleeding due to portal hypertension in CFLD.

CF: cystic fibrosis
CFLD: cystic fibrosis liver disease
RCT; randomised controlled trial
UDCA: ursodeoxycholic acid

Figure 1 PRISMA study flow diagram
Figuras y tablas -
Figure 1

Figure 1 PRISMA study flow diagram

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