Successful treatment with 4-phenylbutyrate in a patient with benign recurrent intrahepatic cholestasis type 2 refractory to biliary drainage and bilirubin absorption

Improved Liver Function and Relieved Pruritus after 4-Phenylbutyrate Therapy in a Patient with Progressive Familial Intrahepatic Cholestasis Type 2

(2014) Sotaro Naoi et al.   JOURNAL OF PEDIATRICS

Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1

(2014) Yasuhiro Hasegawa et al.   Orphanet Journal of Rare Diseases

Treatment of pruritus with Prometheus dialysis and absorption system in a patient with benign recurrent intrahepatic cholestasis

(2013) Urszula Ołdakowska-Jedynak et al.   HEPATOLOGY RESEARCH

Biliary phosphatidylcholine and lysophosphatidylcholine profiles in sclerosing cholangitis

(2013) Annika Gauss   WORLD JOURNAL OF GASTROENTEROLOGY

Characterization of urinary bile acids in a pediatric BRIC-1 patient: Effect of rifampicin treatment

(2012) Tatsuki Mizuochi et al.   CLINICA CHIMICA ACTA

AP2 adaptor complex mediates bile salt export pump internalization and modulates its hepatocanalicular expression and transport function

(2012) Hisamitsu Hayashi et al.   HEPATOLOGY

Successful mutation-specific chaperone therapy with 4-phenylbutyrate in a child with progressive familial intrahepatic cholestasis type 2

(2012) Emmanuel Gonzales et al.   JOURNAL OF HEPATOLOGY

4-Phenylbutyrate modulates ubiquitination of hepatocanalicular MRP2 and reduces serum total bilirubin concentration

(2012) Hisamitsu Hayashi et al.   JOURNAL OF HEPATOLOGY

Benign recurrent intrahepatic cholestasis: review and long-term follow-up of five cases

(2012) Geir Folvik et al.   SCANDINAVIAN JOURNAL OF GASTROENTEROLOGY

Familial cholestasis: Progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis and intrahepatic cholestasis of pregnancy

(2011) Wendy L. van der Woerd et al.   BEST PRACTICE & RESEARCH IN CLINICAL GASTROENTEROLOGY

Lysophosphatidic Acid Is a Potential Mediator of Cholestatic Pruritus

(2010) Andreas E. Kremer et al.   GASTROENTEROLOGY

Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate

(2009) Lieke M. van der Velden et al.   HEPATOLOGY

Progressive familial intrahepatic cholestasis

(2009) Anne Davit-Spraul et al.   Orphanet Journal of Rare Diseases

Successful Treatment with Colestimide for a Bout of Cholestasis in a Japanese Patient with Benign Recurrent Intrahepatic Cholestasis Caused by ATP8B1 Mutation

(2008) Satoko Uegaki et al.   INTERNAL MEDICINE

Phenotypic differences in PFIC2 and BRIC2 correlate with protein stability of mutant Bsep and impaired taurocholate secretion in MDCK II cells

(2007) Tatehiro Kagawa et al.   AMERICAN JOURNAL OF PHYSIOLOGY-GASTROINTESTINAL AND LIVER PHYSIOLOGY