Kidney Function in People With Cystic Fibrosis in the Era of HEMT

April 27, 2026 updated by: Agnieszka Swiatecka-Urban, University of Virginia
The purpose of this study is to find out what causes kidney disease in people with CF. The investigators will study biomarkers in the blood and urine that can either predict who is at risk or detect kidney damage early before it becomes permanent. The study will compare these markers in people with CF over time and during the treatment of lung flare-ups. It will also compare the blood and urine samples obtained from people without CF. The comparison aims to better understand the impact of cystic fibrosis and its treatment on the kidneys, as well as to develop improved methods for preventing, diagnosing, and treating kidney issues associated with CF.

Study Overview

Status

Recruiting

Conditions

Detailed Description

The prevalence of chronic kidney disease is significantly increased in patients with cystic fibrosis (PwCF) with a major impact on morbidity and medication tolerance as people age. Although expressed in both the proximal and distal tubules, the specific contribution of CFTR dysfunction to renal disease remains uncertain. PwCF often are exposed to renal toxins such as frequent aminoglycosides, systemic inflammation, and activated leukocytes, but it is unknown if CFTR dysfunction predisposes to amplified tubular injury. Conventional measures of kidney function, such as serum creatinine, are insensitive to detecting early injury, limiting an opportunity to prevent CKD. This study will address the gaps in early detection and mechanisms of renal dysfunction in CF. The investigators will define the triggers and targetable mechanistic pathways of kidney injury in CF and discover novel strategies for renal protection. The central hypothesis of this study is that CFTR dysfunction alters renal development and increases the inflammatory and fibrogenic responses to nephrotoxic stimuli.

The study involves prospective evaluation of biospecimens (blood and urine) and clinical data. The study analyzes biospecimens in CF outpatients (n=110), CF inpatients (n=110), and healthy subjects (n=40). In the outpatient cohort, biospecimens will be collected at the time of each routine care visit every 3 months for 24 months. PwCF admitted for intravenous (IV) antibiotics will have biospecimens collected on admission and 2/week thereafter during the admission, and then after hospital discharge at each subsequent clinical encounter for 24 months.

These biospecimens will be analyzed for biomarkers, fibrogenic analysis, inflammatory signals, and extracellular vesicles. Clinical data will be examined from chart review and correlated with biospecimen result.

Study Type

Observational

Enrollment (Estimated)

260

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

Study Locations

  • United States
    • Alabama
      • Birmingham, Alabama, United States, 35233
        • Recruiting
        • University of Alabama at Birmingham
        • Contact:
    • New Hampshire
      • Lebanon, New Hampshire, United States, 03756
        • Recruiting
        • Dartmouth-Hitchcock Geisel School of Medicine at Dartmouth
        • Contact:
        • Principal Investigator:
          • Alix Ashare, MD
    • Virginia
      • Charlottesville, Virginia, United States, 22908
        • Recruiting
        • University of Virginia Children's Hospital
        • Contact:

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

  • Child
  • Adult
  • Older Adult

Accepts Healthy Volunteers

Yes

Sampling Method

Non-Probability Sample

Study Population

Participants will be derived from patients with CF with ongoing care at one of the 3 CF Centers (Dartmouth, UAB, UVA).

Description

Inclusion Criteria:

  1. Outpatient CF Cohort

    • Diagnoses of Cystic Fibrosis
    • Age > 30 years old
    • Able to provide informed consent

  2. Inpatient CF Cohort

    • Diagnoses of Cystic Fibrosis
    • Age > 7 years old
    • Able to provide informed consent and assent (where applicable)
    • 55 PwCF frequently hospitalized for a pulmonary exacerbation (>1 hospital admission in the prior 12 months)
    • 55 PwCF sporadically hospitalized for a pulmonary exacerbation (no hospital admissions in the prior 12 months)
    • Able to provide urine sample independently

  3. Healthy Controls

    • Healthy, as per participant self-report
    • Age between 30-50 years
    • Able to provide informed consent

Exclusion Criteria:

  1. Outpatient CF Cohort

    • History of any organ transplant
    • History of immunodeficiency
    • Previous or current cancer diagnoses
    • Pregnant or breastfeeding
    • On chronic dialysis
    • Non-compliance (demonstrated by <2 visits during the 12 months before enrollment)

  2. Inpatient CF Cohort

    • The initiation of intravenous antibiotic therapy after hospital admission before obtaining the first blood and urine sample
    • History of any organ transplant
    • History of immunodeficiency
    • Previous or current cancer diagnoses
    • Pregnant or breastfeeding
    • On chronic dialysis

  3. Healthy Controls

    • History or current kidney disease, organ transplantation, cancer, or any other chronic illness
    • Current use of antibiotics
    • Urinary symptoms or UTI (dysuria, frequency, urgency)
    • Pregnant women
    • Menstruating on the study visit day
    • Blood relatives of PwCF

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

Cohorts and Interventions

Group / Cohort
Outpatient CF Cohort
Not hospitalized CF group: Diagnosis of CF, age >30 y, with ongoing care at one of the 3 CF Centers (Dartmouth, UAB, UVA).
Inpatient CF Cohort
Hospitalized CF cohort: Diagnosis of CF, age >7 y, being admitted for intravenous antibiotic treatment of pulmonary exacerbation.
Healthy Controls
Healthy Volunteers without CF

What is the study measuring?

Primary Outcome Measures

Outcome Measure
Measure Description
Time Frame
Examine whether trajectories of eGFR (calculated from serum creatinine and cystatin C) correlate with urinary kidney injury signatures detected in different urine fractions, or urinary neutrophil levels/activation.
Time Frame: Enrollment and every 3 months for 24 months
Outpatient CF Cohort
Enrollment and every 3 months for 24 months
Correlation between recurrent hospitalizations and urinary kidney injury signature.
Time Frame: On admission before the initiation of intravenous antibiotic therapy, every 48 hrs during the hospitalization, after discharge at each subsequent routine CF care visit for 24 months.
Inpatient CF Cohort
On admission before the initiation of intravenous antibiotic therapy, every 48 hrs during the hospitalization, after discharge at each subsequent routine CF care visit for 24 months.
Relationship between recurrent hospitalization and change in slope of eGFR
Time Frame: On admission before the initiation of intravenous antibiotic therapy, every 48 hrs during the hospitalization, after discharge at each subsequent routine CF care visit for 24 months.
Inpatient CF Cohort
On admission before the initiation of intravenous antibiotic therapy, every 48 hrs during the hospitalization, after discharge at each subsequent routine CF care visit for 24 months.

Secondary Outcome Measures

Outcome Measure
Measure Description
Time Frame
The correlation between changes in the urinary protein biomarker panel, neutrophil activation, and extracellular vesicles over time and eGFR.
Time Frame: Enrollment and every 3 months for 24 months.
Outpatient CF Cohort
Enrollment and every 3 months for 24 months.
Correlation between %FEV1 at admission or decline in %FEV1 and urinary kidney injury signature.
Time Frame: On admission before the initiation of intravenous antibiotic therapy, every 48 hrs during the hospitalization, after discharge at each subsequent routine CF care visit for 24 months.
Inpatient CF Cohort
On admission before the initiation of intravenous antibiotic therapy, every 48 hrs during the hospitalization, after discharge at each subsequent routine CF care visit for 24 months.

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

University of Virginia

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

January 9, 2025

Primary Completion (Estimated)

December 1, 2027

Study Completion (Estimated)

December 1, 2027

Study Registration Dates

First Submitted

September 10, 2024

First Submitted That Met QC Criteria

September 10, 2024

First Posted (Actual)

September 19, 2024

Study Record Updates

Last Update Posted (Actual)

May 4, 2026

Last Update Submitted That Met QC Criteria

April 27, 2026

Last Verified

April 1, 2026

More Information

Terms related to this study

Additional Relevant MeSH Terms

Other Study ID Numbers

  • HSR231650
  • 005245A123 (Other Grant/Funding Number: Cystic Fibrosis Foundation)

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

NO

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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