Ultrasound Elastography to Quantify Liver Disease Severity in Autosomal Recessive Polycystic Kidney Disease.

TitleUltrasound Elastography to Quantify Liver Disease Severity in Autosomal Recessive Polycystic Kidney Disease.
Publication TypeJournal Article
Year of Publication2019
AuthorsHartung EA, Wen J, Poznick L, Furth SL, Darge K
JournalJ Pediatr
Volume209
Pagination107-115.e5
Date Published2019 06
ISSN1097-6833
KeywordsAdolescent, Child, Child, Preschool, Comorbidity, Cross-Sectional Studies, Elasticity Imaging Techniques, Female, Genetic Diseases, Inborn, Hospitals, Pediatric, Humans, Hypertension, Portal, Liver Cirrhosis, Male, Philadelphia, Polycystic Kidney, Autosomal Recessive, Reference Values, ROC Curve, Sensitivity and Specificity, Severity of Illness Index, Ultrasonography, Doppler
Abstract

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.

DOI10.1016/j.jpeds.2019.01.055
Alternate JournalJ. Pediatr.
PubMed ID30902421
PubMed Central IDPMC6535353
Grant ListK23 DK109203 / DK / NIDDK NIH HHS / United States
KL2 TR000139 / TR / NCATS NIH HHS / United States
UL1 RR024134 / RR / NCRR NIH HHS / United States
UL1 TR000003 / TR / NCATS NIH HHS / United States