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References from Congenital hepatic fibrosis and related ciliopathies across the life course: current management, disease burden, and unmet needs in the era of genomic diagnosis and translational therapeutics. Local targets link to admitted publications; unresolved targets remain external evidence.
Congenital hepatic fibrosis: case report and review of literature
10.11604/pamj.2021.38.188.27941 · 2021 · External reference
An update on ductal plate malformations and fibropolycystic diseases of the liver
10.1016/j.humpath.2022.06.022 · 2023 · External reference
Congenital hepatic fibrosis
10.1093/oxfordjournals.qjmed.a066935 · 1961 · External reference
The ductal plate from the inside out: an illustrated review of fibropolycystic liver disease
10.1053/j.sult.2022.06.008 · 2022 · External reference
Elderly onset congenital hepatic fibrosis with portal hypertension diagnosed after recurrent cholangitis: a case report
10.1007/s12328-022-01620-w · 2022 · External reference
Congenital hepatic fibrosis and its mimics: a clinicopathologic study of 19 cases at a single institution
10.1186/s13000-021-01142-y · 2021 · External reference
Fibrocystic liver disease: novel concepts and translational perspectives
10.21037/tgh-2020-04 · 2021 · External reference
Living donor liver transplantation for congenital hepatic fibrosis in children
10.1111/pin.12917 · 2020 · External reference
Biallelic loss-of-function ZFYVE19 mutations are associated with congenital hepatic fibrosis, sclerosing cholangiopathy and high-GGT cholestasis
10.1136/jmedgenet-2019-106706 · 2021 · External reference
Loss of Anks6 leads to YAP deficiency and liver abnormalities
10.1093/hmg/ddaa197 · 2020 · External reference
Kinesin family member 12-related hepatopathy: a generally indolent disorder with elevated gamma-glutamyl-transferase activity
10.1111/cge.14524 · 2024 · External reference
Hepatic phenotypes of HNF1B gene mutations: a case of neonatal cholestasis requiring portoenterostomy and literature review
10.3748/wjg.v21.i8.2550 · 2015 · External reference
Dysregulation of the scribble/YAP/β-catenin axis sustains the fibroinflammatory response in a PKHD1(-/-) mouse model of congenital hepatic fibrosis
10.1096/fj.202101924r · 2022 · External reference
Congenital hepatic fibrosis in a young boy with congenital hypothyroidism: a case report
10.12998/wjcc.v9.i6.1475 · 2021 · External reference
Case report: rare genetic liver disease—a case of congenital hepatic fibrosis in adults with autosomal dominant polycystic kidney disease
10.3389/fmed.2024.1344151 · 2024 · External reference
Clinical features of different clinical forms of childhood congenital hepatic fibrosis
10.7499/j.issn.1008-8830.2016.04.011 · 2016 · External reference
Congenital hepatic fibrosis: clinical presentation, laboratory features and management at a tertiary care hospital of lahore
2016 · External reference
Congenital hepatic fibrosis in children and adults: clinical manifestations, management, and outcome-case series and literature review
10.1155/2020/8284274 · 2020 · External reference
A case of Caroli’s disease confirmed by pathology, atypical symptoms and images
10.3760/cma.j.cn501113-20191020-00384 · 2021 · External reference
Is portal vein cavernous transformation a component of congenital hepatic fibrosis?
10.3748/wjg.v13.i13.1928 · 2007 · External reference
A case report of intrahepatic bile duct dilatation caused by WDR19 gene mutation and presented as Caroli syndrome
10.21037/tp-23-574 · 2024 · External reference
An unusual case of congenital hepatic fibrosis with retinitis pigmentosa
10.7759/cureus.5788 · 2019 · External reference
Isolated congenital hepatic fibrosis associated with TMEM67 mutations: report of a new genotype-phenotype relationship
10.1002/ccr3.981 · 2017 · External reference
Congenital hepatic fibrosis and/or autosomal recessive polycystic kidney disease: a single-center experience
10.5223/pghn.2026.29.1.55 · 2026 · External reference
Characteristics of congenital hepatic fibrosis in a large cohort of patients with autosomal recessive polycystic kidney disease
10.1053/j.gastro.2012.09.056 · 2013 · External reference
A clinical review of congenital hepatic fibrosis diagnosed in adulthood: presentation, complications, and outcomes
10.1016/j.aohep.2023.101164 · 2024 · External reference
Caroli’s syndrome: an early presentation
10.7759/cureus.11029 · 2020 · External reference
Clinical and pathological features of 20 cases of congenital hepatic fibrosis
10.3760/cma.j.cn501113-20231107-00180 · 2023 · External reference
Congenital hepatic fibrosis: CT findings in 18 adults
10.1148/radiol.2311030108 · 2004 · External reference
Congenital cystic lesions of the bile ducts: imaging-based diagnosis
10.1067/j.cpradiol.2019.04.005 · 2020 · External reference
Caroli’s syndrome
10.1055/a-1024-4526 · 2020 · External reference
Imaging of fibropolycystic liver disease
10.1007/s00261-022-03565-7 · 2022 · External reference
Hepatic morphology abnormalities: beyond cirrhosis
10.1007/s00261-017-1351-9 · 2018 · External reference
Unexplained portal hypertension and confusion in an elderly patient: a late presentation of congenital hepatic fibrosis
10.51821/88.1.13474 · 2025 · External reference
Biliary sepsis complication with congenital hepatic fibrosis: an unexpected outcome
10.1186/s12879-023-08681-3 · 2023 · External reference
A rare case mimicking congenital hepatic fibrosis
10.1053/j.gastro.2024.04.018 · 2024 · External reference
Clinicopathological features of congenital hepatic fibrosis
10.3760/cma.j.cn112151-20220725-00653 · 2022 · External reference
Congenital hepatic fibrosis as an early sign of presentation of ADPKD
10.2478/bjmg-2022-0024 · 2023 · External reference
A case report of autosomal recessive polycystic kidney disease with noncompaction of ventricular myocardium: coincidence or different manifestations of ciliopathy?
10.1186/s12882-024-03642-7 · 2024 · External reference
Baveno VII—renewing consensus in portal hypertension
10.1016/j.jhep.2021.12.022 · 2022 · External reference
Investigation report on endoscopic management of esophagogastric variceal bleeding by Chinese endoscopists
10.1097/md.0000000000031263 · 2022 · External reference
Clinical presentation, management, and outcomes for noncirrhotic portal hypertension in children
10.1002/jpn3.70318 · 2026 · External reference
Laparoscopic distal splenoadrenal shunt for the treatment of portal hypertension in children with congenital hepatic fibrosis: a case report
10.1097/md.0000000000005843 · 2017 · External reference
Congenital hepatic fibrosis with negative endoscopic evaluation of esophageal and gastric varices: a case report
10.1097/md.0000000000038424 · 2024 · External reference
Pathogenesis of thrombocytopenia in chronic HCV infection: a review
10.14218/jcth.2020.00007 · 2020 · External reference
Splenectomy improves liver fibrosis via tumor necrosis factor superfamily 14 (LIGHT) through the JNK/TGF-β1 signaling pathway
10.1038/s12276-021-00574-2 · 2021 · External reference
Optimal timing for TIPS and PSE combination treatment in patients with cirrhosis-related variceal bleeding and hypersplenism
10.1016/j.acra.2024.09.003 · 2025 · External reference
Left-sided portal hypertension: what an interventional radiologist can offer?
10.1007/s00330-024-11196-3 · 2025 · External reference
An updated meta-analysis of partial splenic embolization versus splenectomy in the treatment of hypersplenism due to cirrhosis
10.1080/13645706.2021.1933535 · 2022 · External reference
A teenage patient with autosomal recessive polycystic kidney disease, a splenorenal shunt, and congenital hepatic fibrosis: a case report
10.1590/2175-8239-jbn-2018-0081 · 2019 · External reference
Ascites: diagnosis and management
10.1016/j.mcna.2009.03.007 · 2009 · External reference
Diagnostic and therapeutic abdominal paracentesis
10.5694/mja2.51795 · 2023 · External reference
Liver cirrhosis following oxaliplatin-based adjuvant chemotherapy for rectal cancer
10.1177/10781552231178685 · 2023 · External reference
Splenic artery embolization in a patient with advanced Caroli’s syndrome complicated by portal hypertension: a case report
10.1186/s13256-025-05516-4 · 2025 · External reference
Response-Guided therapy with cefotaxime, ceftriaxone, or ciprofloxacin for spontaneous bacterial peritonitis: a randomized trial: a validation study of 2021 AASLD practice guidance for SBP
10.14309/ajg.0000000000002126 · 2023 · External reference
Childhood liver cirrhosis in a resource-limited area: a case report
10.1016/j.ijscr.2025.111549 · 2025 · External reference
Spontaneous bacterial peritonitis: an indication for liver transplantation?
10.3109/00365529609094729 · 1996 · External reference
Congenital hepatic fibrosis and autosomal recessive polycystic kidney disease
10.1097/mpg.0b013e31824711b · 2012 · External reference
Recent progress in the etiopathogenesis of pediatric biliary disease, particularly Caroli’s disease with congenital hepatic fibrosis and biliary atresia
10.14670/hh-25.223 · 2010 · External reference
Tokyo Guidelines 2018: initial management of acute biliary infection and flowchart for acute cholangitis
10.1002/jhbp.509 · 2018 · External reference
Presepsin as a biomarker for risk stratification for acute cholangitis in emergency department: a single-center study
10.12998/wjcc.v9.i32.9857 · 2021 · External reference
Clinical and microbiological characteristics of patients with biliary disease
10.3748/wjg.v26.i14.1638 · 2020 · External reference
Bile culture may guide antibiotic stewardship in acute bacterial cholangitis
10.1007/s10620-024-08289-w · 2024 · External reference
Risk factors for acute cholangitis caused by Enterococcus faecalis and Enterococcus faecium
10.5009/gnl20214 · 2021 · External reference
Is meropenem as a monotherapy truly incompetent for meropenem-nonsusceptible bacterial strains? A pharmacokinetic/pharmacodynamic modeling with monte carlo simulation
10.3389/fmicb.2019.02777 · 2019 · External reference
Is ampicillin plus cephalosporins a therapeutic option for ampicillin-susceptible Enterococcus faecium?
10.1093/jac/dkaf226 · 2025 · External reference
β-Lactams enhance daptomycin activity against vancomycin-resistant Enterococcus faecalis and Enterococcus faecium in in vitro pharmacokinetic/pharmacodynamic models
10.1128/aac.00053-1 · 2015 · External reference
Navigating antibiotic therapy in acute cholangitis: best practices and new insights
10.1002/jhbp.12087 · 2025 · External reference
Antimicrobial therapy of 3 days or less is sufficient after successful ERCP for acute cholangitis
10.1177/2050640620915016 · 2020 · External reference
Antibiotic therapy of 3 days may be sufficient after biliary drainage for acute cholangitis: a systematic review
10.1007/s10620-020-06820-3 · 2021 · External reference
A case of congenital hepatic fibrosis presented with recurrent acute cholangitis
10.4166/kjg.2009.54.6.404 · 2009 · External reference
Oral nonabsorbable antibiotics for prevention of recurrent cholangitis; a brief report study
10.1007/s15010-025-02491-2 · 2025 · External reference
Shewanella: an important, emerging and lethal pathogen in a patient with recurrent presentations of cholangitis
10.1136/bcr-2020-237655 · 2020 · External reference
Outcome of a “step-up approach” for recurrent cholangitis in patients with a non-stenotic hepaticojejunostomy after hepato-pancreato-biliary surgery: single center series
10.1016/j.hpb.2024.10.016 · 2025 · External reference
Congenital cystic diseases of the intra and extrahepatic bile ducts
10.1016/s0399-8320(05)86364-x · 2005 · External reference
Endoscopic management of difficult biliary stones: an evergreen issue
10.3390/medicina60020340 · 2024 · External reference
Peroral cholangioscopy: update on the state-of-the-art
10.4253/wjge.v14.i2.63 · 2022 · External reference
Digital single-operator cholangioscopy in diagnostic and therapeutic bilio-pancreatic diseases: a prospective, multicenter study
10.1016/j.dld.2022.04.019 · 2022 · External reference
Assessing the utility, findings, and outcomes of percutaneous transhepatic cholangioscopy with spyglass(TM) direct visualization system: a case series
10.21037/tgh.2019.11.11 · 2020 · External reference
Large common bile duct stone: a case report
10.1093/jscr/rjaf264 · 2025 · External reference
Repeated balloon dilatation with long-term biliary drainage for treatment of benign biliary-enteric anastomosis strictures: a STROBE-compliant article
10.1097/md.0000000000022741 · 2020 · External reference
Percutaneous transhepatic treatment of benign bile duct strictures using retrievable covered stents: long-term outcomes in 148 patients
10.3348/kjr.2022.0204 · 2022 · External reference
Efficacy of a modified short fully covered self-expandable metal stent for perihilar benign biliary strictures
10.1111/jgh.15258 · 2021 · External reference
Endoscopic stent placement above the sphincter of oddi for biliary strictures after living donor liver transplantation
10.1186/s12876-020-01226-x · 2020 · External reference
Biliary reconstruction for intrahepatic bile duct strictures and optimal jejunal limb length in congenital biliary dilation surgery: a literature review and consensus statements from the AOB consensus meeting
10.1111/ases.70204 · 2026 · External reference
Liver transplantation for congenital hepatic fibrosis
10.1111/tri.13884 · 2021 · External reference
Congenital hepatic fibrosis and need for liver transplantation
2010 · External reference
Congenital hepatic fibrosis leading to cirrhosis and hepatocellular carcinoma: a case report
10.1186/1752-1947-5-160 · 2011 · External reference
Indicators of hepatopulmonary syndrome in patients with portal hypertension. Its various aetiologies, clinical presentations and outcome
2021 · External reference
Prospective evaluation of kidney and liver disease in autosomal recessive polycystic kidney disease-congenital hepatic fibrosis
10.1016/j.ymgme.2020.08.006 · 2020 · External reference
Outcomes of combined liver-kidney transplantation—single center experience
10.1016/j.transproceed.2024.03.014 · 2024 · External reference
Simultaneous liver and kidney transplant in a middle-income country: a single-center experience
10.12659/aot.947649 · 2025 · External reference
Combined liver-kidney transplantation in pediatric patients
10.1111/petr.14666 · 2024 · External reference
Optimal transplant strategy of pediatric liver transplantation for fibropolycystic liver disease: multicenter retrospective study in Japan
10.1111/hepr.14122 · 2024 · External reference
Health related quality of life among rheumatic fever and rheumatic heart disease patients in India
10.1371/journal.pone.0259340 · 2021 · External reference
Pediatric non-cirrhotic portal hypertension: endoscopic outcome and perspectives from developing nations
10.4254/wjh.v13.i10.1269 · 2021 · External reference
Recombinant human growth hormone therapy in autosomal recessive polycystic kidney disease
10.1007/s00467-002-0986-z · 2003 · External reference
Healthcare and economic burden of heart failure with amyloidosis: an insight from national readmission database
10.1016/j.amjms.2023.08.002 · 2023 · External reference
Cellular and molecular features of senescence in acute lung injury
10.1016/j.mad.2020.111410 · 2021 · External reference
Unresolved reference
2023 · External reference
Animal and organoid models of liver fibrosis
10.3389/fphys.2021.666138 · 2021 · External reference
Multi-omics analysis of host-microbiome interactions in a mouse model of congenital hepatic fibrosis
10.1186/s12866-025-03892-x · 2025 · External reference
Cellular mechanisms of liver fibrosis
10.3389/fphar.2021.671640 · 2021 · External reference
The role of hepatic microenvironment in hepatic fibrosis development
10.1080/07853890.2022.2132418 · 2022 · External reference
Oxytocin alleviates liver fibrosis via hepatic macrophages
10.1016/j.jhepr.2024.101032 · 2024 · External reference
Redox biology and liver fibrosis
10.3390/ijms25010410 · 2023 · External reference
Phloridzin reveals new treatment strategies for liver fibrosis
10.3390/ph15070896 · 2022 · External reference
Extracellular vesicles: emerging therapeutic agents for liver fibrosis
10.20517/evcna.2025.08 · 2025 · External reference