ArticleGastroenterology research and practice2020
Congenital Hepatic Fibrosis in Children and Adults: Clinical Manifestations, Management, and Outcome-Case Series and Literature Review.
Article in Gastroenterology research and practice, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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21 citing papers in PubMed, 28 citations in OpenAlex.
- Autosomal dominant polycystic kidney disease in children and adolescents.Childhood kidney diseases · 2026Review
- 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
- Rare liver diseases - Etiology, diagnosis and management: A review.Biomolecules & biomedicine · 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
- Terlipressin-Induced Hyponatremic Seizures in a Patient With Congenital Hepatic Fibrosis.HCA healthcare journal of medicine · 2025Article
- Nephronophthisis 13 caused by WDR19 variants with pancytopenia: case report.CEN case reports · 2024Article
- Heritable Chronic Cholestatic Liver Diseases: A Review.Journal of clinical and translational hepatology · 2024Review
- [A case of neonatal liver failure].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2024Article
- [Idiopathic portal hypertension: a case report].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2024Article
- Case report: Rare genetic liver disease - a case of congenital hepatic fibrosis in adults with autosomal dominant polycystic kidney disease.Frontiers in medicine · 2024Article
- NOTCH signalling - a core regulator of bile duct disease?Disease models & mechanisms · 2023Review
- Congenital Hepatic Fibrosis as an Early Sign of Presentation of ADPKD.Balkan journal of medical genetics : BJMG · 2023Article
- Liver fibrosis in children: a comprehensive review of mechanisms, diagnosis, and therapy.Clinical and experimental pediatrics · 2023Article
- Optimization of the emergency endoscopy process for patients with esophagogastric variceal bleeding using failure mode and effect analysis.American journal of translational research · 2023Article
- Congenital Hepatic Fibrosis in a 2-Year-Old Child Presenting with Fever of Unknown Origin.Case reports in pediatrics · 2023Article
- Elderly onset congenital hepatic fibrosis with portal hypertension diagnosed after recurrent cholangitis: a case report.Clinical journal of gastroenterology · 2022Article
- Hepatic manifestations of systemic disease: an imaging-based review.Pediatric radiology · 2022Review
- Two-dimensional (2D) morphologic measurements can quantify the severity of liver disease in children with autosomal recessive polycystic kidney disease (ARPKD).Abdominal radiology (New York) · 2021Article
- Congenital hepatic fibrosis in a young boy with congenital hypothyroidism: A case report.World journal of clinical cases · 2021Article
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
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Abstract
backgroundCongenital hepatic fibrosis is a hereditary fibropolycystic disease caused by ductal plate malformation. It is characterized by portal hypertension, but the manifestations, management, and outcome vary in children and adults. To raise awareness of medical staff, we have comprehensively compared the clinical features of congenital hepatic fibrosis between children and adults.
methodsWe retrospectively enrolled all patients diagnosed with congenital hepatic fibrosis at the Huashan Hospital from August 2015 to August 2017 and analyzed their familial, clinical, laboratory, imaging, treatment, and follow-up data in detail. In addition, we reviewed cases with congenital hepatic fibrosis reported in the past 20 years in China and analyzed them according to the patients' age.
resultsA total of eight patients were diagnosed with congenital hepatic fibrosis in the study, including four children and four adults. The onset age of the children, who suffered from severe complications of portal hypertension and needed liver transplantation, ranged from 1 to 15 years old. The disorder developed in adults aged 26 to 60 years old. Three adults complained of recurrent abnormal liver function at the onset of illness, and they mainly received conservative treatments. The literature review included 30 children and 33 adults. In comparison, hepatomegaly was more common in children than in adults (57%
conclusionsSerious complications of portal hypertension developed in children requiring liver transplantation, while adults often had mild-to-moderate liver injuries upon onset. Adults with CHF varied a lot in clinical manifestations. Multiorgan involvement and unusual course are helpful to make a diagnosis. Timely histological assessment by liver biopsy and multidisciplinary cooperation are crucial for definitive diagnosis and early intervention.
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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.