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Updated: Jul 31, 2026
07:35
Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring
Published on: June 23, 2015
Autosomal recessive polycystic kidney disease: radiologic-pathologic correlation
G J Lonergan1, R R Rice, E S Suarez
1Department of Radiology and Nuclear Medicine, Uniformed Services University of the Health Sciences, Bethesda, MD, USA. lonergan@afip.osd.mil
Summary
Autosomal recessive polycystic kidney disease (ARPKD) causes kidney and liver cysts, leading to variable symptoms. Management focuses on supportive care, hypertension control, and treatments for renal or liver failure.
Area of Science:
- Nephrology
- Hepatology
- Genetics
Background:
- Autosomal recessive polycystic kidney disease (ARPKD) is a genetic disorder with significant phenotypic variability.
- It is characterized by ectasia and malformation of renal collecting ducts and hepatic biliary ducts, accompanied by fibrosis in both organs.
Purpose of the Study:
- To describe the key clinical and imaging features of ARPKD.
- To outline the spectrum of renal and hepatic involvement and their inverse relationship.
- To summarize current management strategies for ARPKD.
Main Methods:
- The abstract summarizes established knowledge on ARPKD, drawing from clinical observations and imaging findings.
- It reviews the typical presentation and progression of the disease.
Main Results:
- Kidney involvement includes dilated collecting ducts and fibrosis, potentially leading to impaired function, hypertension, and renal failure.
- Liver involvement features biliary duct malformation, fibrosis, and potential portal hypertension, with associated splenomegaly and varices.
- A key finding is the inverse relationship between renal and hepatic disease severity.
Conclusions:
- ARPKD presents with a wide range of kidney and liver manifestations.
- Management is primarily supportive, focusing on symptom control and addressing complications like hypertension and organ failure.
- Dialysis, transplantation, and portal hypertension management are critical interventions for advanced disease.
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