Disease: Cholestasis- benign recurrent intrahepatic 1
- 3D-reconstruction and heterotopic implantation of reduced size monosegment or left lateral segment grafts in small infants: A new technique in pediatric living donor liver transplantation to overcome large-for-size syndrome
- A bi-allelic missense change c.638A > G in matrix metalloproteinase 15 in a patient with progressive familial intrahepatic cholestasis without cardiac anomalies
- A case of progressive familial intrahepatic cholestasis (type 3) as an initial manifestation of cirrhosis-related gastrointestinal bleeding
- A female of progressive familial intrahepatic cholestasis type 3 caused by heterozygous mutations of ABCB4 gene and her cirrhosis improved after treatment of ursodeoxycholic acid: a case report
- A homozygous R148W mutation in Semaphorin 7A causes progressive familial intrahepatic cholestasis
- ABCB11 deficiency presenting as transient neonatal cholestasis: Correlation with genotypes and BSEP expression
- ABCB4 Mutations in Adults Cause a Spectrum Cholestatic Disorder Histologically Distinct from Other Biliary Disease
- 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 Deficiency Results in Elevated Mitochondrial Phosphatidylethanolamine Levels and Increased Mitochondrial Oxidative Phosphorylation in Human Hepatoma Cells
- ATP8B1, ABCB11, and ABCB4 Genes Defects: Novel Mutations Associated with Cholestasis with Different Phenotypes and Outcomes
- Benign biliary strictures treated with biodegradable stents in patients with surgically altered anatomy using double balloon enteroscopy
- 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
- Biliary diversion in progressive familial intrahepatic cholestasis: a systematic review and meta-analysis
- Burden of illness of progressive familial intrahepatic cholestasis in the US, UK, France, and Germany: study rationale and protocol of the PICTURE study
- Case Report: A Novel Homozygous Variant Identified in a Chinese Patient With Benign Recurrent Intrahepatic Cholestasis-Type 1
- Case Report: A Rare Case of Benign Recurrent Intrahepatic Cholestasis-Type 1 With a Novel Heterozygous Pathogenic Variant of <em>ATP8B1</em>
- Case Report: A Rare Heterozygous <em>ATP8B1</em> Mutation in a BRIC1 Patient: Haploinsufficiency?
- Childhood Cholestatic Liver Diseases that Persist Into Adulthood: Lessons for the Adult Gastroenterologist
- Cholestasis Due to USP53 Deficiency
- Clinical and genetic analysis of cases of progressive familial intrahepatic cholestasis type 3
- Clinical and genetic characterization of pediatric patients with progressive familial intrahepatic cholestasis type 3 (PFIC3): identification of 14 novel ABCB4 variants and review of the literatures
- Clinical features, diagnosis, and treatment strategies of progressive familial intrahepatic cholestasis
- Clinical symptoms, biochemistry, and liver histology during the native liver period of progressive familial intrahepatic cholestasis type 2
- Coexistence of Three Different Mutations in a Male Infant: neurofibromatosis Type 1, Progressive Familial Intrahepatic Cholestasis Type 2 and LPIN3
- Compound Heterozygous MYO5B Mutation, a Cause of Infantile Cholestasis: A Case Report
- Dental management of a pediatric patient with progressive familial intrahepatic cholestasis having dental anomalies: a case report and brief review of the literature
- Development of the Patient- and Observer-Reported PRUCISION Instruments to Assess Pruritus and Sleep Disturbance in Pediatric Patients with Cholestatic Liver Diseases
- Diagnosis and management of Alagille and progressive familial intrahepatic cholestasis
- Disease Course and Treatment Response of Eosinophilic Gastrointestinal Diseases in Children With Liver Transplantation: Long-Term Follow-Up
- Dual β-Catenin and γ-Catenin Loss in Hepatocytes Impacts Their Polarity through Altered Transforming Growth Factor-β and Hepatocyte Nuclear Factor 4α Signaling
- Early Disseminated Mycobacterium Abscessus Complex Infection in an Infant with Coexisting Cystic Fibrosis and Progressive Familial Intrahepatic Cholestasis: Case report and literature review
- Effect of food on the pharmacokinetics and therapeutic efficacy of 4-phenylbutyrate in progressive familial intrahepatic cholestasis
- Effects of partial internal biliary diversion on long-term outcomes in patients with progressive familial intrahepatic cholestasis: experience in 44 patients
- Epidemiology and burden of progressive familial intrahepatic cholestasis: a systematic review
- Expression of miR-let7b and miR-19b in progressive familial intrahepatic cholestasis (PFIC) children
- Extrahepatic manifestations of progressive familial intrahepatic cholestasis syndromes: Presentation of a case series and literature review
- Extremity deformity due to intractable pruritus and resolution with liver transplantation in progressive familial intrahepatic cholestasis
- Gene Therapy for Progressive Familial Intrahepatic Cholestasis: Current Progress and Future Prospects
- Glycerol Phenylbutyrate Therapy in Progressive Familial Intrahepatic Cholestasis Type 2
- Heterozygous mutations of ATP8B1, ABCB11 and ABCB4 cause mild forms of Progressive Familial Intrahepatic Cholestasis in a pediatric cohort
- Ileal bypass for pruritus relief in a 3-year-old boy with advanced progressive familial intrahepatic cholestasis: how effective is it?
- Ileal exclusion for pruritus treatment in children with progressive familial intrahepatic cholestasis and other cholestatic diseases
- Impact of Genotype, Serum Bile Acids, and Surgical Biliary Diversion on Native Liver Survival in FIC1 Deficiency
- Impact of progressive familial intrahepatic cholestasis on caregivers: caregiver-reported outcomes from the multinational PICTURE study
- Improvement of cholestatic episodes in patients with benign recurrent intrahepatic cholestasis (BRIC) treated with rifampicin. A long-term follow-up
- Letter to the editor: The burgeoning field of progressive familial intrahepatic cholestasis
- Liver Steatosis and Diarrhea After Liver Transplantation for Progressive Familial Intrahepatic Cholestasis Type 1: Can Biliary Diversion Solve These Problems?
- Long-Term Colestyramine Treatment Prevents Cholestatic Attacks in Refractory Benign Recurrent Intrahepatic Cholestasis Type 1 Disease
- Long-term liver transplant outcomes for progressive familial intrahepatic cholestasis type 1: The Pittsburgh experience
- Long-Term Outcome after Liver Transplantation for Progressive Familial Intrahepatic Cholestasis
- Long-term outcome following cholecystocolostomy in 41 patients with progressive familial intrahepatic cholestasis
- Long-term Outcomes of Living-donor Liver Transplantation for Progressive Familial Intrahepatic Cholestasis Type 1
- Long-Term Outcomes of Patients With Progressive Familial Intrahepatic Cholestasis After Biliary Diversion
- Management and outcomes after liver transplantation for progressive familial intrahepatic cholestasis: A systematic review and meta-analysis
- Maralixibat for the treatment of PFIC: Long-term, IBAT inhibition in an open-label, Phase 2 study
- Maralixibat: First Approval
- Molecular and computational characterization of ABCB11 and ABCG5 variants in Tunisian patients with neonatal/infantile low-GGT intrahepatic cholestasis: Genetic diagnosis and genotype-phenotype correlation assessment
- Molecular overview of progressive familial intrahepatic cholestasis
- Nasobiliary drainage prior to surgical biliary diversion in progressive familial intrahepatic cholestasis type II
- Native liver survival in bile salt export pump deficiency: results of a retrospective cohort study
- Neonatal-onset Progressive Familial Intrahepatic Cholestasis (PFIC): first molecular study in Tunisian patients
- NR1H4 disease: rapidly progressing neonatal intrahepatic cholestasis and early death
- NR1H4-related Progressive Familial Intrahepatic Cholestasis 5: Further Evidence for Rapidly Progressive Liver Failure
- Odevixibat (Bylvay) for progressive familial intrahepatic cholestasis-associated pruritus
- Odevixibat treatment in progressive familial intrahepatic cholestasis: a randomised, placebo-controlled, phase 3 trial
- Odevixibat: a promising new treatment for progressive familial intrahepatic cholestasis
- Optimal liver transplant procedure in progressive familial intrahepatic cholestasis type 1 treated with biliary diversion or intestinal transplantation: Lessons learned from three cases treated with different approaches
- Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis
- Overview of Progressive Familial Intrahepatic Cholestasis
- Oxysterol/chitotriosidase based selective screening for Niemann-Pick type C in infantile cholestasis syndrome patients
- Paediatric research sets new standards for therapy in paediatric and adult cholestasis
- Partial Biliary Diversion May Promote Long-Term Relief of Pruritus and Native Liver Survival in Children with Cholestatic Liver Diseases
- Partial External Biliary Diversion for Severe Diarrhea After Liver Transplant in Patients with Progressive Familial Intrahepatic Cholestasis Type 1
- Phenotype and genetic analysis of a pedigree affected with progressive familial intrahepatic cholestasis
- Plectin Mutations in Progressive Familial Intrahepatic Cholestasis
- Positive phase III results for odevixibat for progressive familial intrahepatic cholestasis
- Potential of ileal bile acid transporter inhibition as a therapeutic target in Alagille syndrome and progressive familial intrahepatic cholestasis
- Progressive Familial Intrahepatic Cholestasis Associated With USP53 Gene Mutation in a Brazilian Child
- Progressive Familial Intrahepatic Cholestasis Type 2 and Recurrence After Liver Transplantation: A Case Report
- Progressive familial intrahepatic cholestasis type 4 in an Indian child: presentation, initial course and novel compound heterozygous mutation
- Progressive Familial Intrahepatic Cholestasis: Is It Time to Transition to Genetic Cholestasis?
- Reduced spontaneous itch in mouse models of cholestasis
- Serum Transaminase Monitoring and Successful Treatment of ADHD With Dextroamphetamine in a Patient With Progressive Familial Intrahepatic Cholestasis Type 3: A Case Report and Literature Review
- Severe Neonatal Presentation of Progressive Familial Intrahepatic Cholestasis Type 4 in an Omani Infant
- Severe vitamin deficiencies in pregnancy complicated by progressive familial intrahepatic cholestasis
- Single-center experience in management of progressive familial intrahepatic cholestasis
- Successful treatment of a patient with benign recurrent intrahepatic cholestasis type 1 with albumin dialysis
- Successful Treatment of Patient With Ewing Sarcoma in the Setting of Inherited Cholestatic Liver Disease
- Surgical diversion of enterohepatic circulation in pediatric cholestasis
- Systematic Review and Meta-analysis: Partial External Biliary Diversion in Progressive Familial Intrahepatic Cholestasis
- The First Korean Adult Case of Progressive Familial Intrahepatic Cholestasis Type 7 with Novel <em>USP53</em> Splicing Variants by Next Generation Sequencing
- The first new drug for progressive familial intrahepatic cholestasis
- The molecular landscape of progressive familial intrahepatic cholestasis in Turkey: Defining the molecular profiles and expanding the variant spectrum
- The spectrum of Progressive Familial Intrahepatic Cholestasis diseases: Update on pathophysiology and emerging treatments
- The zonula occludens protein family regulates the hepatic barrier system in the murine liver
- Two novel ATP8B1 mutations involved in progressive familial intrahepatic cholestasis type 1 that is ameliorated by rifampicin: A case report
- Validation of the PRUCISION Instruments in Pediatric Patients with Progressive Familial Intrahepatic Cholestasis
- Whole-Genome Sequencing Reveals Large ATP8B1 Deletion/Duplications as Second Mutations Missed by Exome-Based Sequencing