Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
3293672 | Gastroenterology | 2013 | 12 Pages |
Abstract
Platelet count is the best predictor of the severity of portal hypertension, which has early onset but is underdiagnosed in patients with ARPKD. Seventy percent of patients with ARPKD have biliary abnormalities. Kidney and liver disease are independent, and variability in severity is not explainable by type of PKHD1 mutation; ClinicalTrials.gov number, NCT00068224.
Keywords
GGTI-FABPCBDARPKDChFNIHGFRMRCPγ-glutamyltransferaseMRIAPRiautosomal recessive polycystic kidney diseasemagnetic resonance cholangiopancreatographyMagnetic resonance imagingprothrombin timeUltrasonographyUSGcongenital hepatic fibrosisNational Institutes of Healthcommon bile ductDuctal plate malformationGlomerular filtration rateaspartate aminotransferase to platelet ratio indexPortal hypertensionIntestinal fatty acid binding proteinGeneticsHigh-resolution
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Authors
Meral Gunay-Aygun, Esperanza Font-Montgomery, Linda Lukose, Maya Tuchman Gerstein, Katie Piwnica-Worms, Peter Choyke, Kailash T. Daryanani, Baris Turkbey, Roxanne Fischer, Isa Bernardini, Murat Sincan, Xiongce Zhao, Netanya G. Sandler,