Liver Stiffness in Patients with Underlying Polycystic Kidney Disorders.
J
Jean Molleston, MD
Primary Investigator
Overview
- Use Fibroscan to determine thevalence of CHF in an ADPKD population and the incidence over a four-yeard in these patients.
- Use Fibroscan to determine thevalence of congenital hepatic fibrosis in an ARPKD population
- Use fibroscan to determine thevalence of fibrotic liver disease in patients with other cystic kidneydiseases such as nephronophthisis and other genetic syndromes that includeboth liver and kidney pathology
- Correlate the degree ofgenital hepatic fibrosis, as measured by FibroScan, with laboratorygression to clinical signs of portalhypertension.
Description
- Identify patients in our practice with
cystic kidney diseases using ICD10 codes.
-Perform retrospective chart review.
-Create pedigrees of patients with discovered
fibrosis in ADPKD to better characterize inheritance patterns of CHF in this
population.
-Obtain Fibroscan imaging to classify the
degree of hepatic fibrosis at time of enrollment and every two years for the
next four years
Liver
stiffness <6.9 kPa is indicative of no or minimal fibrosis
Liver
stiffness ≥ 6.9 kPa signifies liver fibrosis
- Collect clinical data including laboratory
studies from the routine clinical care to correlate with the fibroscan
Eligibility
You may be eligible for this study if you meet the following criteria:
-
Conditions:
Polycystic Kidney Disorders
-
Age: Between 3 Years - 65 Years
-
Gender: All
Inclusion Criteria:Phase I: Any pediatric patient, ages 3-25, who has known polycystic kidney disease (regardless of inheritance pattern) or congenital hepatic fibrosis will be eligible for the study.
Phase II: Any adult patient, ages 18-65, who has known polycystic kidney disease (regardless of inheritance pattern) or congenital hepatic fibrosis will be eligible for the study.
Exclusion Criteria:
- Presence of clinically significant ascites detected on physical examination as this limits fibroscan interpretation.
- Open wound near expected FibroScan probe application site.
Updated on
03 May 2024.
Study ID: 2008362785
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