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Imaging Assessments of ARPKD Kidney Disease Progression

Who can join

Ages 6 and older · All sexes · Healthy volunteers welcome

Inclusion criteria:

ALL SUBJECTS

* Ages \>6 years old
* Able to lay still for 30 minutes in an MRI scanner ARPKD Patients
* Clinical and/or genetic diagnosis of ARPKD
* eGFR (creatinine based) \>30ml/min/1.732 performed within the last year Healthy Controls
* Otherwise healthy with no known structural or functional kidney disease
* No history of hypertension or documented high blood pressure within the last year

Exclusion criteria:

ALL SUBJECTS

* Contraindications to MRI (e.g. metal implants)
* Parental/patient refusal to sign an informed consent/ assent form ARPKD Patients
* Prior kidney or other solid organ transplant
* eGFR \<30ml/min/1.732 Healthy Controls
* Systemic diseases known to place patient at risk for kidney disease (e.g. diabetes mellitus)
* Obesity (BMI \>95th percentile for age for \<18 years old, or BMI\>30 for adults)
* History of prematurity (\<32 weeks gestational age)

About the study

Autosomal Recessive Polycystic Kidney Disease (ARPKD) is a potentially lethal genetic disorder for which there are currently no disease specific treatments. Clinical trials have been limited by the absence of robust measures of disease progression. The overall goal of this 5 year NIH-funded prospective, observational multicenter study conducted at CC (primary site) and Children's Hospital of Philadelphia (CHOP) (collaborating site) is establish a set of rapid, quantitative, and reproducible novel kidney MRI measures applicable to standard clinical MRI scanners, which could serve as potential biomarkers to measure response to therapy in patients across the disease spectrum and all ages.

The investigators will recruit a total of 45 ARPKD patients and 15 healthy controls at the the two sites . All subjects will be \> or = 6yrs old with no contraindications to undergoing MRI (non contrast). ARPKD subjects will be recruited into one of 3 cohorts based on their estimated glomerular filtration rate (eGFR): early CKD (eGFR\> or =90ml/min/1.73m2), mild CKD (60-89ml/min/1.73m2) and moderate CKD (30-59ml/min/1.73m2). For ARPKD subjects, participation will last 3 years and consist of a baseline and 3 subsequent annual visits. Healthy controls will only have 1 study visit. Study visits for all participants will include collection of clinical and demographic data, clinical blood and urine tests (E.g. serum creatinine) and MRI imaging. ARPKD (but not healthy subjects) will also undergo a measured GFR test (iohexol clearance) to accurately assess their kidney function.

Sponsor: The Cleveland Clinic · Participants: 60 · Started: Dec 12, 2024

Contact the study team

• Katherine M Dell, MD · Phone: 216-444-6123
• Research Coordinator · Phone: 216-386-9360

Official record on ClinicalTrials.gov — NCT07201025

Locations in the U.S.

From ClinicalTrials.gov, data retrieved Sep 30, 2026. Each study sets its own eligibility; the study team decides who can join.