Related Experiment Video
Updated: Aug 14, 2026
05:56
Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
Published on: August 29, 2025
The hepatic fibrocystic diseases
Nanda Kerkar1, Karen Norton, Frederick J Suchy
1Department of Pediatrics, Mount Sinai School of Medicine, 1 Gustave L. Levy Place, New York, NY 10029, USA.
Clinics in Liver Disease
|December 27, 2005
Summary
Hepatic fibrocystic diseases involve biliary abnormalities and can be linked to polycystic kidney diseases. Management focuses on supportive care and addressing complications, with liver transplantation as a potential option.
Area of Science:
- Hepatology
- Genetics
- Nephrology
Background:
- Hepatic fibrocystic diseases encompass a spectrum of intrahepatic biliary abnormalities.
- These conditions can manifest alongside autosomal recessive or dominant polycystic kidney diseases.
Purpose of the Study:
- To describe the clinical spectrum and management of hepatic fibrocystic diseases.
- To highlight the association with renal cystic diseases and treatment strategies.
Main Methods:
- Review of clinical presentations.
- Analysis of associated biliary and renal abnormalities.
- Evaluation of treatment outcomes.
Main Results:
- Variable intrahepatic biliary abnormalities including fibrosis and cystic formations.
- Association with polycystic kidney diseases and renal collecting system dilatation.
- Symptoms often arise from fibrosis or dilated ducts/cysts, leading to complications like infection and lithiasis.
Conclusions:
- Hepatic fibrocystic diseases require careful management, focusing on supportive care and associated renal disease.
- Liver transplantation is a viable option for select patients with severe hepatic involvement.
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