ReviewTranslational gastroenterology and hepatology2021
Fibrocystic liver disease: novel concepts and translational perspectives.
Review in Translational gastroenterology and hepatology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 34 citations in OpenAlex.
- Congenital hepatic fibrosis in an adult female combined with Von Meyenburg complex and autosomal dominant polycystic kidney disease: a case report.Frontiers in gastroenterology (Lausanne, Switzerland) · 2026Article
- Congenital Hepatic Fibrosis and/or Autosomal Recessive Polycystic Kidney Disease: A Single-center Experience.Pediatric gastroenterology, hepatology & nutrition · 2026Article
- 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.Frontiers in pediatrics · 2026Review
- Clinical outcomes of liver transplantation in Caroli syndrome: a retrospective analysis.Orphanet journal of rare diseases · 2025Article
- Polycystic Kidney Disease in Children: The Current Status and the Next Horizon.American journal of kidney diseases : the official journal of the National Kidney Foundation · 2025Review
- [A case of congenital hepatic fibrosis accompanied with multiple system abnormalities].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2025Article
- Heritable Chronic Cholestatic Liver Diseases: A Review.Journal of clinical and translational hepatology · 2024Review
- Biliary fibrosis is an important but neglected pathological feature in hepatobiliary disorders: from molecular mechanisms to clinical implications.Medical review (2021) · 2024Review
- [A case of cholangitis-type congenital hepatic fibrosis due to a mutation in the polycystic kidney/hepatic disease 1 gene].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2024Article
- An Approach to Investigations of Chronic Liver Disease.Indian journal of pediatrics · 2024Review
- Histopathology and molecular pathology confirmed a diagnosis of atypical Caroli's syndrome: a case report.Diagnostic pathology · 2024Article
- Exceptional Liver Transplant Indications: Unveiling the Uncommon Landscape.Diagnostics (Basel, Switzerland) · 2024Review
- Biliary sepsis complication with congenital hepatic fibrosis: an unexpected outcome.BMC infectious diseases · 2023Article
- Imaging of fibropolycystic liver disease.Abdominal radiology (New York) · 2022Review
- Hepatic manifestations of systemic disease: an imaging-based review.Pediatric radiology · 2022Review
- Vitamin D supplementation for autoimmune hepatitis: A need for further investigation.World journal of hepatology · 2022Article
- Polycystic Liver Disease: Advances in Understanding and Treatment.Annual review of pathology · 2022Review
- Inflammatory pathways and cholangiocarcinoma risk mechanisms and prevention.Advances in cancer research · 2022Review
- Case report: Severe hepatic fibrosis induced by chronic cholestasis of congenital biliary dilation treated by laparoscopic surgery after immunonutrition support- An infantile case.Frontiers in pediatrics · 2022Article
- Article
Corrections and comments
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Authors and funding
5 authors at 3 institutions in 2 countries.
Funding
Abstract
Fibrocystic liver diseases (FLDs) comprise a heterogeneous group of rare diseases of the biliary tree, having in common an abnormal development of the embryonic ductal plate caused by genetically-determined dysfunctions of proteins expressed in the primary cilia of cholangiocytes (and therefore grouped among the "ciliopathies"). The ductal dysgenesis may affect the biliary system at multiple levels, from the small intrahepatic bile ducts [congenital hepatic fibrosis (CHF)], to the larger intrahepatic bile ducts [Caroli disease (CD), or Caroli syndrome (CS), when CD coexists with CHF], leading to biliary microhamartomas and segmental bile duct dilations. Biliary changes are accompanied by progressive deposition of abundant peribiliary fibrosis. Peribiliary fibrosis and biliary cysts are the fundamental lesions of FLDs and are responsible for the main clinical manifestations, such as portal hypertension, recurrent cholangitis, cholestasis, sepsis and eventually cholangiocarcinoma. Furthermore, FLDs often associate with a spectrum of disorders affecting primarily the kidney. Among them, the autosomal recessive polycystic kidney disease (ARPKD) is the most frequent, and the renal function impairment is central in disease progression. CHF, CD/CS, and ARPKD are caused by a number of mutations in polycystic kidney hepatic disease 1 (
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.