Liver Stiffness in Patients with Underlying Polycystic Kidney Disorders.
J
Jean Molleston, MD
Primary Investigator
Enrolling By Invitation
3 years - 65 years
All
Phase
N/A
100 participants needed
1 Location
Brief description of study
- 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.
Detailed description of study
- 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 of study
You may be eligible for this study if you meet the following criteria:
- Conditions: Polycystic Kidney Disorders, Riley
-
Age: 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
13 Sep 2024.
Study ID: 2008362785
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