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Xu, R. Progressive Familial Intrahepatic Cholestasis. Encyclopedia. Available online: https://encyclopedia.pub/entry/5466 (accessed on 21 September 2026).
Xu R. Progressive Familial Intrahepatic Cholestasis. Encyclopedia. Available at: https://encyclopedia.pub/entry/5466. Accessed September 21, 2026.
Xu, Rita. "Progressive Familial Intrahepatic Cholestasis" Encyclopedia, https://encyclopedia.pub/entry/5466 (accessed September 21, 2026).
Xu, R. (2020, December 24). Progressive Familial Intrahepatic Cholestasis. In Encyclopedia. https://encyclopedia.pub/entry/5466
Xu, Rita. "Progressive Familial Intrahepatic Cholestasis." Encyclopedia. Web. 24 December, 2020.
Progressive Familial Intrahepatic Cholestasis
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This entry is adapted from the peer-reviewed paper https://medlineplus.gov/genetics/condition/progressive-familial-intrahepatic-cholestasis

Progressive familial intrahepatic cholestasis (PFIC) is a disorder that causes progressive liver disease, which typically leads to liver failure. In people with PFIC, liver cells are less able to secrete a digestive fluid called bile. The buildup of bile in liver cells causes liver disease in affected individuals.

genetic conditions

References

  1. Alissa FT, Jaffe R, Shneider BL. Update on progressive familial intrahepaticcholestasis. J Pediatr Gastroenterol Nutr. 2008 Mar;46(3):241-52. doi:10.1097/MPG.0b013e3181596060. Review.
  2. Davit-Spraul A, Gonzales E, Baussan C, Jacquemin E. Progressive familialintrahepatic cholestasis. Orphanet J Rare Dis. 2009 Jan 8;4:1. doi:10.1186/1750-1172-4-1. Review.
  3. Harris MJ, Le Couteur DG, Arias IM. Progressive familial intrahepaticcholestasis: genetic disorders of biliary transporters. J Gastroenterol Hepatol. 2005 Jun;20(6):807-17. Review.
  4. Jansen PL, Sturm E. Genetic cholestasis, causes and consequences forhepatobiliary transport. Liver Int. 2003 Oct;23(5):315-22. Review.
  5. Knisely AS, Bull LN, Shneider BL. ATP8B1 Deficiency. 2001 Oct 15 [updated 2014Mar 20]. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, Bean LJH, Stephens K,Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University ofWashington, Seattle; 1993-2020. Available fromhttp://www.ncbi.nlm.nih.gov/books/NBK1297/
  6. Pauli-Magnus C, Stieger B, Meier Y, Kullak-Ublick GA, Meier PJ. Enterohepatic transport of bile salts and genetics of cholestasis. J Hepatol. 2005Aug;43(2):342-57. Review.
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Subjects: Genetics & Heredity
Contributor MDPI registered users' name will be linked to their SciProfiles pages. To register with us, please refer to https://encyclopedia.pub/register : Rita Xu
View Times: 849
Entry Collection: MedlinePlus
Revision: 1 time (View History)
Update Date: 24 Dec 2020
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