Related Experiment Video
Updated: Jan 27, 2026
A Possible Zebrafish Model of Polycystic Kidney Disease: Knockdown of wnt5a Causes Cysts in Zebrafish Kidneys
Published on: December 2, 2014
Ultrasound Elastography to Quantify Liver Disease Severity in Autosomal Recessive Polycystic Kidney Disease
Erum A Hartung1, Jessica Wen2, Laura Poznick3
1Division of Nephrology, Children's Hospital of Philadelphia, Philadelphia, PA; Department of Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA.
Insights
Ultrasound elastography accurately detects liver fibrosis and portal hypertension in children with autosomal recessive polycystic kidney disease (ARPKD). This noninvasive method is especially effective using the left liver lobe to identify these conditions.
Area of Science:
- Pediatric Nephrology
- Hepatology
- Medical Imaging
Background:
- Autosomal recessive polycystic kidney disease (ARPKD) is a genetic disorder often associated with congenital hepatic fibrosis and portal hypertension.
- Accurate noninvasive methods are needed to assess liver complications in children with ARPKD.
- Ultrasound elastography, specifically Acoustic Radiation Force Impulse (ARFI) imaging, offers a promising approach for evaluating liver stiffness.
Purpose of the Study:
- To evaluate the diagnostic accuracy of ultrasound ARFI elastography for detecting congenital hepatic fibrosis and portal hypertension in children with ARPKD.
- To assess the utility of ARFI elastography in differentiating ARPKD patients from healthy controls and in identifying portal hypertension within the ARPKD cohort.
Main Methods:
- A cross-sectional study involving 25 children with ARPKD and 24 healthy controls.
- Ultrasound ARFI elastography was performed to measure shear wave speed (SWS) in the liver and spleen.
- Statistical analyses included comparisons between groups, correlation analyses, and receiver operating characteristic (ROC) analysis to determine diagnostic accuracy.
Main Results:
- Children with ARPKD exhibited significantly higher liver and spleen SWS compared to controls.
- The left liver lobe demonstrated high diagnostic accuracy (AUC 0.92) with 92% sensitivity and 96% specificity for distinguishing ARPKD patients.
- ARPKD patients with portal hypertension showed significantly increased liver and spleen stiffness compared to those without portal hypertension.
Conclusions:
- Ultrasound ARFI elastography is a valuable noninvasive biomarker for detecting and quantifying liver fibrosis in children with ARPKD.
- The technique, particularly measurements from the left liver lobe, effectively identifies portal hypertension in this pediatric population.
- ARFI elastography provides a reliable, noninvasive tool for monitoring liver complications in children diagnosed with ARPKD.
Objectives:
To evaluate the diagnostic accuracy of ultrasound elastography with acoustic radiation force impulse (ARFI) to detect congenital hepatic fibrosis and portal hypertension in children with autosomal recessive polycystic kidney disease (ARPKD).
Study Design:
Cross-sectional study of 25 children with ARPKD and 24 healthy controls. Ultrasound ARFI elastography (Acuson S3000, Siemens Medical Solutions USA, Inc, Malvern, Pennsylvania) was performed to measure shear wave speed (SWS) in the right and left liver lobes and the spleen. Liver and spleen SWS were compared in controls vs ARPKD, and ARPKD without vs with portal hypertension. Linear correlations between liver and spleen SWS, spleen length, and platelet counts were analyzed. Receiver operating characteristic analysis was used to evaluate diagnostic accuracy of ultrasound ARFI elastography.
Results:
Participants with ARPKD had significantly higher median liver and spleen SWS than controls. At a proposed SWS cut-off value of 1.56 m/s, the left liver lobe had the highest sensitivity (92%) and specificity (96%) for distinguishing participants with ARPKD from controls (receiver operating characteristic area 0.92; 95% CI 0.82-1.00). Participants with ARPKD with portal hypertension (splenomegaly and low platelet counts) had significantly higher median liver and spleen stiffness than those without portal hypertension. The left liver lobe also had the highest sensitivity and specificity for distinguishing subjects with ARPKD with portal hypertension.
Conclusions:
Ultrasound ARFI elastography of the liver and spleen, particularly of the left liver lobe, is a useful noninvasive biomarker to detect and quantify liver fibrosis and portal hypertension in children with ARPKD.
More Related Videos
05:56Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
Published on: August 29, 2025
07:35Use 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
Related Concept Videos
01:26Diseases of the Liver and Gallbladder
Cirrhosis is characterized by the scarring of hepatic lobules in the liver, which are replaced by fibrous tissue, affecting the liver's normal functioning. NAFLD, on the other hand, is caused by an excessive build-up of fat in the liver, not...
01:25Chronic Kidney Disease I: Introduction
01:24Chronic Kidney Disease II: Clinical Manifestations
01:28Chronic Kidney Disease III: Interprofessional Care
01:18Chronic Kidney Disease IV: Nursing Management
01:22Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test