Disease: Cholestasis- benign recurrent intrahepatic 2
- Bile salt export pump deficiency disease: two novel, late onset, ABCB11 mutations identified by next generation sequencing
- Hepatic immunohistochemistry of bile transporters in progressive familial intrahepatic cholestasis
- A Drug Regimen for Progressive Familial Cholestasis Type 2
- A novel mutation within a transmembrane helix of the bile salt export pump (BSEP, ABCB11) with delayed development of cirrhosis
- A Physiology-Based Model of Human Bile Acid Metabolism for Predicting Bile Acid Tissue Levels After Drug Administration in Healthy Subjects and BRIC Type 2 Patients
- A Rare BSEP Mutation Associated with a Mild Form of Progressive Familial Intrahepatic Cholestasis Type 2
- ABCB11 deficiency presenting as transient neonatal cholestasis: Correlation with genotypes and BSEP expression
- Antisense oligonucleotides rescue an intronic splicing variant in the ABCB11 gene that causes progressive familial intrahepatic cholestasis type 2
- Assessment of Adenosine Triphosphatase Phospholipid Transporting 8B1 (ATP8B1) Function in Patients With Cholestasis With ATP8B1 Deficiency by Using Peripheral Blood Monocyte-Derived Macrophages
- ATP8B1, ABCB11, and ABCB4 Genes Defects: Novel Mutations Associated with Cholestasis with Different Phenotypes and Outcomes
- Autoimmune BSEP disease: disease recurrence after liver transplantation for progressive familial intrahepatic cholestasis
- Balancing Medical and Non-Accidental Causes of Multiple Fractures in a Child with Progressive Familial Intrahepatic Cholestasis
- Benign biliary strictures treated with biodegradable stents in patients with surgically altered anatomy using double balloon enteroscopy
- Benign recurrent intrahepatic cholestasis - 2 (BRIC-2)/ABCB11 deficiency in a young child - Report from a tertiary care center in South India
- Benign recurrent intrahepatic cholestasis (BRIC)-like episode associated with ATP8B1 variation underlying protracted cholestatic course of acute hepatitis E virus infection
- Benign Recurrent Intrahepatic Cholestasis Type 1 with Novel Nonsense Mutations in the <em>ATP8B1</em> Gene
- Benign recurrent intrahepatic cholestasis type 2 in a child: A case report and novel mutation
- Benign Recurrent Intrahepatic Cholestasis Type 2 in Siblings with Novel <em>ABCB11</em> Mutations
- Beyond an Obvious Cause of Cholestasis in a Toddler: Compound Heterozygosity for <em>ABCB11</em> Mutations
- Bile acid pool dynamics in progressive familial intrahepatic cholestasis with partial external bile diversion
- Bile salt export pump-reactive antibodies form a polyclonal, multi-inhibitory response in antibody-induced bile salt export pump deficiency
- Biosynthesis and trafficking of the bile salt export pump, BSEP: therapeutic implications of BSEP mutations
- Brucella infection in a child with progressive familial intrahepatic cholestasis type 2 who had undergone liver transplantation
- Burden of illness of progressive familial intrahepatic cholestasis in the US, UK, France, and Germany: study rationale and protocol of the PICTURE study
- Can genetic testing guide the therapy of cholestatic pruritus? A case of benign recurrent intrahepatic cholestasis type 2 with severe nasobiliary drainage-refractory itch
- Case Report: A Novel Single Variant TJP2 Mutation in a Case of Benign Recurrent Intrahepatic Cholestasis
- Cholestasis After Pediatric Liver Transplantation-Recurrence of a Progressive Familial Intrahepatic Cholestasis Phenotype as a Rare Differential Diagnosis: A Case Report
- Cholestasis in Benign Recurrent Intrahepatic Cholestasis 2
- Clinical phenotype and molecular analysis of a homozygous ABCB11 mutation responsible for progressive infantile cholestasis
- Clinical symptoms, biochemistry, and liver histology during the native liver period of progressive familial intrahepatic cholestasis type 2
- Clinical utility gene card for: progressive familial intrahepatic cholestasis type 2
- Clinical Variability After Partial External Biliary Diversion in Familial Intrahepatic Cholestasis 1 Deficiency
- Comorbidity between progressive familial intrahepatic cholestasis and atopic dermatitis in a 19-month-old child
- Cryptogenic cholestasis in young and adults: ATP8B1, ABCB11, ABCB4, and TJP2 gene variants analysis by high-throughput sequencing
- Diagnosis of ABCB11 gene mutations in children with intrahepatic cholestasis using high resolution melting analysis and direct sequencing
- Diagnostic Yield of an Algorithm for Neonatal and Infantile Cholestasis Integrating Next-Generation Sequencing
- Early Diagnosis of <em>ABCB11</em> Spectrum Liver Disorders by Next Generation Sequencing
- Elevated copper impairs hepatic nuclear receptor function in Wilson's disease
- Endoscopic Balloon Dilation for Benign Bilioenteric Stricture: Outcomes and Factors Affecting Recurrence
- Endoscopic nasobiliary drainage improves jaundice attack symptoms in benign recurrent intrahepatic cholestasis: A case report
- Exon-skipping and mRNA decay in human liver tissue: molecular consequences of pathogenic bile salt export pump mutations
- From Laparoscopic Cholecystectomy to Liver Transplantation: When the Gallbladder Becomes the Pandora s Box
- Genetic contribution of ABCC2 to Dubin-Johnson syndrome and inherited cholestatic disorders
- Genetic Testing in Liver Disease: What to Order, in Whom, and When
- Genetics and Molecular Modeling of New Mutations of Familial Intrahepatic Cholestasis in a Single Italian Center
- Hepatotoxicity from anabolic androgenic steroids marketed as dietary supplements: contribution from ATP8B1/ABCB11 mutations?
- High affinity anti-BSEP antibodies after liver transplantation for PFIC-2 - Successful treatment with immunoadsorption and B-cell depletion
- Ileal exclusion in children with progressive familial intrahepatic cholestasis
- Importance of Reverse Translational Research (rTR)
- Improved liver function and relieved pruritus after 4-phenylbutyrate therapy in a patient with progressive familial intrahepatic cholestasis type 2
- In Vitro Rescue of the Bile Acid Transport Function of ABCB11 Variants by CFTR Potentiators
- Liver transcript analysis reveals aberrant splicing due to silent and intronic variations in the ABCB11 gene
- Long-term balloon indwelling technique for the treatment of single benign biliary stricture
- Long-term follow-up in children with progressive familial intrahepatic cholestasis type 2 after partial external biliary diversion with focus on histopathological features
- Long-term therapy of a patient with summerskill-walshe-tygstrup syndrome by applying prometheus® liver dialysis: a case report
- Management and outcomes after liver transplantation for progressive familial intrahepatic cholestasis: A systematic review and meta-analysis
- Massive gene amplification drives paediatric hepatocellular carcinoma caused by bile salt export pump deficiency
- Minimal-invasive Cholecystocolostomy as Surgical Bridging Procedure in Patients with Progressive Familial Intrahepatic Cholestasis (PFIC)
- Molecular Genetic Dissection and Neonatal/Infantile Intrahepatic Cholestasis Using Targeted Next-Generation Sequencing
- New Insights in Genetic Cholestasis: From Molecular Mechanisms to Clinical Implications
- New paradigms of USP53 disease: normal GGT cholestasis, BRIC, cholangiopathy, and responsiveness to rifampicin
- Nonsense variant of <em>ATP8B1</em> gene in heterozygosis and benign recurrent intrahepatic cholestasis: A case report and review of literature
- Odevixibat and partial external biliary diversion showed equal improvement of cholestasis in a patient with progressive familial intrahepatic cholestasis
- One case of benign recurrent intrahepatic cholestasis type 2
- Outcomes following partial external biliary diversion in patients with progressive familial intrahepatic cholestasis
- Paediatric hepatocellular carcinoma due to somatic CTNNB1 and NFE2L2 mutations in the setting of inherited bi-allelic ABCB11 mutations
- Partial external biliary diversion in bile salt export pump deficiency: Association between outcome and mutation
- Pathophysiology and Diseases of the Proximal Pathways of the Biliary System
- Pediatric Pure Red Cell Aplasia Caused by Tacrolimus After Living-Donor Liver Transplant
- Pharmacological Premature Termination Codon Readthrough of ABCB11 in Bile Salt Export Pump Deficiency: An In Vitro Study
- Phenotype-Genotype Correlation of North Indian Progressive Familial Intrahepatic Cholestasis type2 Children Shows p.Val444Ala and p.Asn591Ser Variants and Retained BSEP Expression
- Phospholipase D2 mediates signaling by ATPase class I type 8B membrane 1
- Polymorphisms in ABCB11 and ATP8B1 Associated with Development of Severe Intrahepatic Cholestasis in Hodgkin's Lymphoma
- Post-transplant Recurrent Bile Salt Export Pump Disease: A Form of Antibody-mediated Graft Dysfunction and Utilization of C4d
- Progressive familial intrahepatic cholestasis
- Progressive familial intrahepatic cholestasis
- Progressive Familial Intrahepatic Cholestasis Type 2 and Recurrence After Liver Transplantation: A Case Report
- Recurrence of Progressive Familial Intrahepatic Cholestasis Type 2 Phenotype After Living-donor Liver Transplantation: A Case Report
- Retargeting of bile salt export pump and favorable outcome in children with progressive familial intrahepatic cholestasis type 2
- Rituximab as therapy for the recurrence of bile salt export pump deficiency after liver transplantation
- Safety and efficacy of long-term nasobiliary drainage to treat intractable pruritus in cholestatic liver disease
- Serum Autotaxin Activity Correlates With Pruritus in Pediatric Cholestatic Disorders
- Successful mutation-specific chaperone therapy with 4-phenylbutyrate in a child with progressive familial intrahepatic cholestasis type 2
- Successful pregnancy after ileal exclusion in progressive familial intrahepatic cholestasis type 2
- Successful treatment with 4-phenylbutyrate in a patient with benign recurrent intrahepatic cholestasis type 2 refractory to biliary drainage and bilirubin absorption
- Successful Treatment with Rituximab and Immunoadsorption for an Auto-Antibody Induced Bile Salt Export Pump Deficiency in a Liver Transplanted Patient
- Surgical diversion of enterohepatic circulation in pediatric cholestasis
- Surgical outcomes in Alagille syndrome and PFIC: A single institution's 20-year experience
- Systematic review of progressive familial intrahepatic cholestasis
- Targeted pharmacotherapy in progressive familial intrahepatic cholestasis type 2: Evidence for improvement of cholestasis with 4-phenylbutyrate
- The bile salt export pump (BSEP) in health and disease
- The Bile Salt Export Pump: Molecular Structure, Study Models and Small-Molecule Drugs for the Treatment of Inherited BSEP Deficiencies
- The lipid flippase heterodimer ATP8B1-CDC50A is essential for surface expression of the apical sodium-dependent bile acid transporter (SLC10A2/ASBT) in intestinal Caco-2 cells
- The spectrum of novel ABCB11 gene variations in children with progressive familial intrahepatic cholestasis type 2 in Pakistani cohorts
- Triggers of benign recurrent intrahepatic cholestasis and its pathophysiology: a review of literature
- Two Case Reports of Successful Treatment of Cholestasis With Steroids in Patients With PFIC-2
- Ursodeoxycholic acid stabilizes the bile salt export pump in the apical membrane in MDCK II cells
- Visceral symptoms as a key diagnostic sign for the early infantile form of Niemann-Pick disease type C in a Russian patient: a case report
- Xenobiotic Nuclear Receptor Signaling Determines Molecular Pathogenesis of Progressive Familial Intrahepatic Cholestasis
- Zebrafish abcb11b mutant reveals strategies to restore bile excretion impaired by bile salt export pump deficiency