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Cholestasis and cholestatic syndromes.
O'Leary JG, Pratt DS. O'Leary JG, et al. Curr Opin Gastroenterol. 2007 May;23(3):232-6. doi: 10.1097/MOG.0b013e3280d942d8. Curr Opin Gastroenterol. 2007. PMID: 17414837 Review.
RECENT FINDINGS: Highlights include new discoveries for the role of the farsenoid X receptor and sodium-dependent taurocholate cotransporting polypeptide; new insights into the pathogenesis of progressive familial intrahepatic cholestasis type …
RECENT FINDINGS: Highlights include new discoveries for the role of the farsenoid X receptor and sodium-dependent taurocholate cotransportin …
Partial External Biliary Diversion for Severe Diarrhea After Liver Transplant in Patients with Progressive Familial Intrahepatic Cholestasis Type 1.
Teker Düztaş D, Sarı S, Eğritaş Gürkan Ö, Sözen MH, Dalgıç B, Dalgıç A. Teker Düztaş D, et al. Exp Clin Transplant. 2022 May;20(Suppl 3):81-84. doi: 10.6002/ect.PediatricSymp2022.O27. Exp Clin Transplant. 2022. PMID: 35570607 Free article.
A biliary diversion operation for disruption of enterohepatic circulation in patients with progressive familial intrahepatic cholestasis type 1 without cirrhosis is another option. We present a pediatric patient with progressive …
A biliary diversion operation for disruption of enterohepatic circulation in patients with progressive familial intrahepati …
Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1.
Hasegawa Y, Hayashi H, Naoi S, Kondou H, Bessho K, Igarashi K, Hanada K, Nakao K, Kimura T, Konishi A, Nagasaka H, Miyoshi Y, Ozono K, Kusuhara H. Hasegawa Y, et al. Orphanet J Rare Dis. 2014 Jul 15;9:89. doi: 10.1186/1750-1172-9-89. Orphanet J Rare Dis. 2014. PMID: 25022842 Free PMC article.
BACKGROUND: Progressive familial intrahepatic cholestasis type 1 (PFIC1), an inherited liver disease caused by mutations in ATP8B1, progresses to severe cholestasis with a sustained intractable itch. ...METHODS: The therapeutic potency of …
BACKGROUND: Progressive familial intrahepatic cholestasis type 1 (PFIC1), an inherited liver disea …
Living Donor Liver Transplantation for Progressive Familial Intrahepatic Cholestasis Type 1: Two Reported Cases.
Oya Y, Sugawara Y, Honda M, Yoshii D, Isono K, Hayashida S, Yamamoto H, Inomata Y. Oya Y, et al. Transplant Proc. 2017 Jun;49(5):1123-1125. doi: 10.1016/j.transproceed.2017.03.035. Transplant Proc. 2017. PMID: 28583540
BACKGROUND: Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an inherited disease characterized by cholestatic features. ...
BACKGROUND: Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an inherited disease c …
Successful Treatment of Patient With Ewing Sarcoma in the Setting of Inherited Cholestatic Liver Disease.
Daley J, Halligan K, Howrie D, Salgado CM, Superdock A, Friehling E, Bailey KM. Daley J, et al. J Pediatr Hematol Oncol. 2023 Jul 1;45(5):e621-e623. doi: 10.1097/MPH.0000000000002623. Epub 2023 Jan 12. J Pediatr Hematol Oncol. 2023. PMID: 36728269 Free PMC article.
Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an inherited, progressive cholestatic liver disease. ...
Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an inherited, progressive cholesta …
Progressive familial intrahepatic cholestasis type 1 and extrahepatic features: no catch-up of stature growth, exacerbation of diarrhea, and appearance of liver steatosis after liver transplantation.
Lykavieris P, van Mil S, Cresteil D, Fabre M, Hadchouel M, Klomp L, Bernard O, Jacquemin E. Lykavieris P, et al. J Hepatol. 2003 Sep;39(3):447-52. doi: 10.1016/s0168-8278(03)00286-1. J Hepatol. 2003. PMID: 12927934
CONCLUSIONS: Progressive familial intrahepatic cholestasis characterized by normal serum gamma-glutamyltransferase activity and extrahepatic features corresponds to progressive familial intrahepatic cholestasis type 1. Extrahepatic sympto …
CONCLUSIONS: Progressive familial intrahepatic cholestasis characterized by normal serum gamma-glutamyltransferase activity and extrahepatic …
Novel ATP8B1 mutation in an adult male with progressive familial intrahepatic cholestasis.
Deng BC, Lv S, Cui W, Zhao R, Lu X, Wu J, Liu P. Deng BC, et al. World J Gastroenterol. 2012 Nov 28;18(44):6504-9. doi: 10.3748/wjg.v18.i44.6504. World J Gastroenterol. 2012. PMID: 23197899 Free PMC article.
Progressive familial intrahepatic cholestasis type 1 is a rare disease that is characterized by low serum gamma-glutamyltransferase levels due to mutation in ATP8B1. ...
Progressive familial intrahepatic cholestasis type 1 is a rare disease that is characterized by lo …
ATP8B1 and ATP11C: Two Lipid Flippases Important for Hepatocyte Function.
Naik J, de Waart DR, Utsunomiya K, Duijst S, Mok KH, Oude Elferink RP, Bosma PJ, Paulusma CC. Naik J, et al. Dig Dis. 2015;33(3):314-8. doi: 10.1159/000371665. Epub 2015 May 27. Dig Dis. 2015. PMID: 26045263
Recently it was shown that loss of the P4 ATPases ATP8B1 and ATP11C are associated with severe Cholestatic liver disease. Mutation of ATP8B1 cause progressive familial Intrahepatic Cholestasis type 1 (PFIC1)and benign recurrent intrahepat …
Recently it was shown that loss of the P4 ATPases ATP8B1 and ATP11C are associated with severe Cholestatic liver disease. Mutation of ATP8B1 …