Τι είναι η Προϊούσα Οικογενής Ενδοηπατική Χολόσταση (PFIC);
Σχετικά με την PFIC
Τι συμβαίνει στο ήπαρ;
Συχνά συμπτώματα
Διάγνωση και θεραπεία
Η έγκαιρη διάγνωση έχει σημασία
Βιβλιογραφία:
  1. Baker A, et al. 2019. Systematic review of progressive familial intrahepatic cholestasis. Clin Resh Hepatol Gastroenterol. 43(1):20-36.
  2. Nayagam NS, et al. 2022. Clinical phenotype of adult‐onset liver disease in patients with variants in ABCB4, ABCB11, and ATP8B1. Hepatol Commun. 6(10):2654-2664.
  3. A Siddiqi I and Tadi P. 2023. Progressive Familial Intrahepatic Cholestasis. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing. Available at: https://www.ncbi.nlm.nih.gov/books/NBK559317/. Accessed April 2024.
  4. Bansal, Nalini and Mukul Rastogi. 2017. “An Itchy Experience - PFIC 3 Masquerading as Wilson’s Disease; Learning from Mistakes.” Oncology, Gastroenterology and Hepatology Reports. 6(1):67-71.
  5. Davit-Spraul A, et al. 2009. Progressive familial intrahepatic cholestasis. Orphanet J Rare Dis. 4(1):1-12.
  6. AbuBotain H and Khounganian R. 2013. Dental management of a patient with progressive familial intrahepatic cholestasis. King Saud University Journal of Dental Sciences. 4(1):37-45.
  7. Jones-Hughes T, et al. 2021. Epidemiology and burden of progressive familial intrahepatic cholestasis: a systematic review. Orphanet J Rare Dis. 16(1):255.
  8. Chiang JYL. 2013. Bile Acid Metabolism and Signaling. Comprehensive Physiology. 3(3):1191–1212.
  9. Langedijk JAGM, et al. 2021. Cholestasis-Associated Pruritus and Its Pruritogens. Frontiers Medicine. 8(639674):1-13.
  10. Srivastava A. 2014. Progressive Familial Intrahepatic Cholestasis. J Clin Exp Hepatol. 4(1):25-36.
  11. Mehl A, et al. 2016. Liver transplantation and the management of progressive familial intrahepatic cholestasis in children. World J Transplant. 6(2):278-290.
  12. Gunaydin M, et al. 2018. Progressive familial intrahepatic cholestasis: diagnosis, management, and treatment. Hepatic Medicine: Evidence and Research. 10(10):95-104.
  13. Mighiu C, et al. 2022. Impact of progressive familial intrahepatic cholestasis on caregivers: caregiver-reported outcomes from the multinational PICTURE study. Orphanet J Rare Dis. 17(32):1-12.
  14. McKiernan P, et al. 2023. Opinion paper on the diagnosis and treatment of progressive familial intrahepatic cholestasis. Journal of Hepatology Report. 6(1):100949.
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