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Liver Disease Associated with Cystic Fibrosis

MedGen UID:
417687
Concept ID:
C2827436
Disease or Syndrome
Synonym: Cystic Fibrosis Liver Disease

Definition

A spectrum of liver disorders that develop in patients with cystic fibrosis. It includes asymptomatic elevation of the liver function tests, steatosis, cholecystitis with cholelithiasis, sclerosing cholangitis, biliary cirrhosis, and cholangiocarcinoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLiver Disease Associated with Cystic Fibrosis

Professional guidelines

PubMed

Colombo C, Crosignani A, Battezzati PM, Castellani MR, Comi S, Melzi ML, Giunta A
J Hepatol 1999 Oct;31(4):672-7. doi: 10.1016/s0168-8278(99)80347-x. PMID: 10551391

Recent clinical studies

Etiology

Efremova I, Maslennikov R, Poluektova E, Vasilieva E, Zharikov Y, Suslov A, Letyagina Y, Kozlov E, Levshina A, Ivashkin V
World J Gastroenterol 2023 Jun 14;29(22):3400-3421. doi: 10.3748/wjg.v29.i22.3400. PMID: 37389240Free PMC Article
Gana S, Serpieri V, Valente EM
Am J Med Genet C Semin Med Genet 2022 Mar;190(1):72-88. Epub 2022 Mar 3 doi: 10.1002/ajmg.c.31963. PMID: 35238134Free PMC Article
Larson-Nath C, Goday P
Nutr Clin Pract 2019 Jun;34(3):349-358. Epub 2019 Apr 8 doi: 10.1002/ncp.10274. PMID: 30963628
Capurso L
J Clin Gastroenterol 2019 Mar;53 Suppl 1:S1-S41. doi: 10.1097/MCG.0000000000001170. PMID: 30741841
Tojo R, Suárez A, Clemente MG, de los Reyes-Gavilán CG, Margolles A, Gueimonde M, Ruas-Madiedo P
World J Gastroenterol 2014 Nov 7;20(41):15163-76. doi: 10.3748/wjg.v20.i41.15163. PMID: 25386066Free PMC Article

Diagnosis

Gana S, Serpieri V, Valente EM
Am J Med Genet C Semin Med Genet 2022 Mar;190(1):72-88. Epub 2022 Mar 3 doi: 10.1002/ajmg.c.31963. PMID: 35238134Free PMC Article
Goggolidou P, Richards T
Biochim Biophys Acta Mol Basis Dis 2022 Apr 1;1868(4):166348. Epub 2022 Jan 12 doi: 10.1016/j.bbadis.2022.166348. PMID: 35032595
Larson-Nath C, Goday P
Nutr Clin Pract 2019 Jun;34(3):349-358. Epub 2019 Apr 8 doi: 10.1002/ncp.10274. PMID: 30963628
Bachmann-Gagescu R, Dempsey JC, Phelps IG, O'Roak BJ, Knutzen DM, Rue TC, Ishak GE, Isabella CR, Gorden N, Adkins J, Boyle EA, de Lacy N, O'Day D, Alswaid A, Ramadevi A R, Lingappa L, Lourenço C, Martorell L, Garcia-Cazorla À, Ozyürek H, Haliloğlu G, Tuysuz B, Topçu M; University of Washington Center for Mendelian Genomics, Chance P, Parisi MA, Glass IA, Shendure J, Doherty D
J Med Genet 2015 Aug;52(8):514-22. Epub 2015 Jun 19 doi: 10.1136/jmedgenet-2015-103087. PMID: 26092869Free PMC Article
Lazaridis KN, LaRusso NF
Mayo Clin Proc 2015 Jun;90(6):791-800. Epub 2015 May 6 doi: 10.1016/j.mayocp.2015.03.017. PMID: 25957621Free PMC Article

Therapy

Wasuwanich P, Karnsakul W
Minerva Pediatr 2020 Oct;72(5):440-447. Epub 2020 May 15 doi: 10.23736/S0026-4946.20.05895-8. PMID: 32418413
Mallea J, Bolan C, Cortese C, Harnois D
Liver Transpl 2019 Aug;25(8):1265-1275. Epub 2019 Jun 25 doi: 10.1002/lt.25496. PMID: 31102574
Capurso L
J Clin Gastroenterol 2019 Mar;53 Suppl 1:S1-S41. doi: 10.1097/MCG.0000000000001170. PMID: 30741841
Tojo R, Suárez A, Clemente MG, de los Reyes-Gavilán CG, Margolles A, Gueimonde M, Ruas-Madiedo P
World J Gastroenterol 2014 Nov 7;20(41):15163-76. doi: 10.3748/wjg.v20.i41.15163. PMID: 25386066Free PMC Article
Herrmann U, Dockter G, Lammert F
Best Pract Res Clin Gastroenterol 2010 Oct;24(5):585-92. doi: 10.1016/j.bpg.2010.08.003. PMID: 20955961

Prognosis

Efremova I, Maslennikov R, Poluektova E, Vasilieva E, Zharikov Y, Suslov A, Letyagina Y, Kozlov E, Levshina A, Ivashkin V
World J Gastroenterol 2023 Jun 14;29(22):3400-3421. doi: 10.3748/wjg.v29.i22.3400. PMID: 37389240Free PMC Article
Gana S, Serpieri V, Valente EM
Am J Med Genet C Semin Med Genet 2022 Mar;190(1):72-88. Epub 2022 Mar 3 doi: 10.1002/ajmg.c.31963. PMID: 35238134Free PMC Article
Goggolidou P, Richards T
Biochim Biophys Acta Mol Basis Dis 2022 Apr 1;1868(4):166348. Epub 2022 Jan 12 doi: 10.1016/j.bbadis.2022.166348. PMID: 35032595
Wasuwanich P, Karnsakul W
Minerva Pediatr 2020 Oct;72(5):440-447. Epub 2020 May 15 doi: 10.23736/S0026-4946.20.05895-8. PMID: 32418413
Mallea J, Bolan C, Cortese C, Harnois D
Liver Transpl 2019 Aug;25(8):1265-1275. Epub 2019 Jun 25 doi: 10.1002/lt.25496. PMID: 31102574

Clinical prediction guides

Olivença DV, Davis JD, Kumbale CM, Zhao CY, Brown SP, McCarty NA, Voit EO
WIREs Mech Dis 2023 Nov-Dec;15(6):e1625. Epub 2023 Aug 6 doi: 10.1002/wsbm.1625. PMID: 37544654Free PMC Article
Westhaus A, Cabanes-Creus M, Dilworth KL, Zhu E, Salas Gómez D, Navarro RG, Amaya AK, Scott S, Kwiatek M, McCorkindale AL, Hayman TE, Frahm S, Perocheau DP, Tran BM, Vincan E, Wong SL, Waters SA, Riddiough GE, Perini MV, Wilson LOW, Baruteau J, Diecke S, González-Aseguinolaza G, Santilli G, Thrasher AJ, Alexander IE, Lisowski L
Hum Gene Ther 2023 Apr;34(7-8):273-288. doi: 10.1089/hum.2022.188. PMID: 36927149Free PMC Article
Colombo C, Alicandro G, Oliver M, Lewindon PJ, Ramm GA, Ooi CY, Alghisi F, Kashirskaya N, Kondratyeva E, Corti F, Padoan R, Asherova I, Evans H, de Monestrol I, Strandvik B, Lindblad A; CF UDCA study group
J Cyst Fibros 2022 Mar;21(2):220-226. Epub 2021 Apr 2 doi: 10.1016/j.jcf.2021.03.014. PMID: 33814323
Bachmann-Gagescu R, Dempsey JC, Phelps IG, O'Roak BJ, Knutzen DM, Rue TC, Ishak GE, Isabella CR, Gorden N, Adkins J, Boyle EA, de Lacy N, O'Day D, Alswaid A, Ramadevi A R, Lingappa L, Lourenço C, Martorell L, Garcia-Cazorla À, Ozyürek H, Haliloğlu G, Tuysuz B, Topçu M; University of Washington Center for Mendelian Genomics, Chance P, Parisi MA, Glass IA, Shendure J, Doherty D
J Med Genet 2015 Aug;52(8):514-22. Epub 2015 Jun 19 doi: 10.1136/jmedgenet-2015-103087. PMID: 26092869Free PMC Article
Dennis CM, McNeil KD, Dunning J, Stewart S, Friend PJ, Alexander G, Higenbottam TW, Calne RY, Wallwork J
J Heart Lung Transplant 1996 May;15(5):536-8. PMID: 8771510

Recent systematic reviews

Caley LR, White H, de Goffau MC, Floto RA, Parkhill J, Marsland B, Peckham DG
Dig Dis Sci 2023 May;68(5):1797-1814. Epub 2023 Jan 4 doi: 10.1007/s10620-022-07812-1. PMID: 36600119
Armaghanian N, Brand-Miller JC, Markovic TP, Steinbeck KS
J Cyst Fibros 2016 May;15(3):274-84. Epub 2016 Mar 22 doi: 10.1016/j.jcf.2016.02.012. PMID: 27025865
Anie KA, Green J
Cochrane Database Syst Rev 2015 May 8;2015(5):CD001916. doi: 10.1002/14651858.CD001916.pub3. PMID: 25966336Free PMC Article
Moser JJ, Veale PM, McAllister DL, Archer DP
Paediatr Anaesth 2013 Nov;23(11):1084-96. Epub 2013 Sep 3 doi: 10.1111/pan.12255. PMID: 24033636
Anie KA, Green J
Cochrane Database Syst Rev 2012 Feb 15;(2):CD001916. doi: 10.1002/14651858.CD001916.pub2. PMID: 22336781

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