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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

Delayed intestinal visualization at hepatobiliary scintigraphy is associated with response to long-term treatment with ursodeoxycholic acid in patients with cystic fibrosis-associated liver disease.
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

Therapy

Cystic fibrosis-associated liver disease in children.
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
Cystic Fibrosis-Associated Liver Disease in Lung Transplant Recipients.
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
Thirty Years of Lactobacillus rhamnosus GG: A Review.
Capurso L
J Clin Gastroenterol 2019 Mar;53 Suppl 1:S1-S41. doi: 10.1097/MCG.0000000000001170. PMID: 30741841
Intestinal microbiota in health and disease: role of bifidobacteria in gut homeostasis.
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
Cystic fibrosis-associated liver disease.
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

International Diabetes Federation Position Statement on the 1-hour post-load plasma glucose for the diagnosis of intermediate hyperglycaemia and type 2 diabetes.
Bergman M, Manco M, Satman I, Chan J, Schmidt MI, Sesti G, Vanessa Fiorentino T, Abdul-Ghani M, Jagannathan R, Kumar Thyparambil Aravindakshan P, Gabriel R, Mohan V, Buysschaert M, Bennakhi A, Pascal Kengne A, Dorcely B, Nilsson PM, Tuomi T, Battelino T, Hussain A, Ceriello A, Tuomilehto J
Diabetes Res Clin Pract 2024 Mar;209:111589. Epub 2024 Mar 7 doi: 10.1016/j.diabres.2024.111589. PMID: 38458916
Epidemiology of small intestinal bacterial overgrowth.
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
Genotype-phenotype correlates in Joubert syndrome: A review.
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
The genetics of Autosomal Recessive Polycystic Kidney Disease (ARPKD).
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
Cystic fibrosis-associated liver disease in children.
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

Clinical prediction guides

International Diabetes Federation Position Statement on the 1-hour post-load plasma glucose for the diagnosis of intermediate hyperglycaemia and type 2 diabetes.
Bergman M, Manco M, Satman I, Chan J, Schmidt MI, Sesti G, Vanessa Fiorentino T, Abdul-Ghani M, Jagannathan R, Kumar Thyparambil Aravindakshan P, Gabriel R, Mohan V, Buysschaert M, Bennakhi A, Pascal Kengne A, Dorcely B, Nilsson PM, Tuomi T, Battelino T, Hussain A, Ceriello A, Tuomilehto J
Diabetes Res Clin Pract 2024 Mar;209:111589. Epub 2024 Mar 7 doi: 10.1016/j.diabres.2024.111589. PMID: 38458916
Mathematical models of cystic fibrosis as a systemic disease.
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
Ursodeoxycholic acid and liver disease associated with cystic fibrosis: A multicenter cohort study.
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
Joubert syndrome: a model for untangling recessive disorders with extreme genetic heterogeneity.
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
Heart-lung-liver transplantation.
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

Macrolide antibiotics (including azithromycin) for cystic fibrosis.
Southern KW, Solis-Moya A, Kurz D, Smith S
Cochrane Database Syst Rev 2024 Feb 27;2(2):CD002203. doi: 10.1002/14651858.CD002203.pub5. PMID: 38411248Free PMC Article
Cystic Fibrosis-Related Gut Dysbiosis: A Systematic Review.
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
Hypoglycaemia in cystic fibrosis in the absence of diabetes: A systematic review.
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
Psychological therapies for sickle cell disease and pain.
Anie KA, Green J
Cochrane Database Syst Rev 2015 May 8;2015(5):CD001916. doi: 10.1002/14651858.CD001916.pub3. PMID: 25966336Free PMC Article
A systematic review and quantitative analysis of neurocognitive outcomes in children with four chronic illnesses.
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

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