Longitudinal Assessment of Transient Elastography in Cystic Fibrosis

NCT ID: NCT03001388

Last Updated: 2025-04-11

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

141 participants

Study Classification

OBSERVATIONAL

Study Start Date

2017-03-08

Study Completion Date

2023-05-09

Brief Summary

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To determine if transient elastography (TE), when combined with ultrasound (US) pattern characterization can improve the prediction of progression to a nodular pattern on US.

To confirm the feasibility of obtaining TE measurements in children with Cystic Fibrosis (CF) To prospectively assess whether TE data are associated with conventional laboratory markers of hepatic fibrosis To determine the variability of TE measurements taken at different sites in the same patient

Detailed Description

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A noninvasive assessment of hepatic fibrosis is desperately needed to advance the care of children with CF significant liver disease and to provide for measurements during clinical trials. That global assessment might serve as both a predictor/descriptor of disease course but also as a critical biomarker for clinical research. FibroScan® measurement of liver stiffness has great potential to fill this void. The underlying hypothesis of this proposal is that elastography in addition to US can improved the prediction of the development of a nodular liver on US and development of portal hypertension over time in children and young adults with CF.

Conditions

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Cystic Fibrosis Liver Disease

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Interventions

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Transient Elastography (TE)

Participants enrolled in Cystic Fibrosis Liver Disease Network (CFLD NET)Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis (PUSH) study in longitudinal follow up at centers with Fibroscan available (currently 8/11 centers)

Intervention Type PROCEDURE

Eligibility Criteria

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Inclusion Criteria

* Participants enrolled in CFLD NET PUSH study in longitudinal follow up at centers with Fibroscan available (currently 8/11 centers)
* Entry criteria for that study were:
* CF as determined by sweat chloride \>60 meq/l
* Pancreatic insufficiency
* Age 3-12 years old at entry
* For entry into the longitudinal follow up subjects were in one of two groups

* A screening US pattern of nodular liver (CIR), heterogeneous increased echogenicity (HTG) or homogeneous increased echogenicity (HMG)
* A screening US pattern of normal (NL) matched to a HTG subject (2 NL:1HTG) by age, center and pseudomonas status

Exclusion Criteria

* Exited from the PUSH Study
* Unable / unwilling to sign consent
Minimum Eligible Age

6 Years

Maximum Eligible Age

26 Years

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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Cystic Fibrosis Foundation

OTHER

Sponsor Role collaborator

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

NIH

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Ed Doo, MD

Role: STUDY_DIRECTOR

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

Averell Sherker, MD

Role: STUDY_DIRECTOR

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)

Locations

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Children's Hospital Colorado

Aurora, Colorado, United States

Site Status

Children's Healthcare of Atlanta

Atlanta, Georgia, United States

Site Status

Ann & Robert H. Lurie Children's

Chicago, Illinois, United States

Site Status

Riley Hospital for Children

Indianapolis, Indiana, United States

Site Status

Cincinnati Children's Hospital Medical

Cincinnati, Ohio, United States

Site Status

Texas Children's Hospital

Houston, Texas, United States

Site Status

Seattle Children's Hospital

Seattle, Washington, United States

Site Status

The Hospital for Sick Children

Toronto, Ontario, Canada

Site Status

Countries

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United States Canada

References

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Ye W, Leung DH, Molleston JP, Ling SC, Murray KF, Nicholas JL, Huang S, Karmazyn BW, Harned RK, Masand P, Alazraki AL, Navarro OM, Otto RK, Palermo JJ, Towbin AJ, Alonso EM, Karnsakul WW, Jane Schwarzenberg S, Seidel GF, Siegel M, Magee JC, Narkewicz MR, Jay Freeman A. Association Between Transient Elastography and Controlled Attenuated Parameter and Liver Ultrasound in Children With Cystic Fibrosis. Hepatol Commun. 2021 May 13;5(8):1362-1372. doi: 10.1002/hep4.1719. eCollection 2021 Aug.

Reference Type BACKGROUND
PMID: 34430781 (View on PubMed)

Related Links

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Other Identifiers

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U01DK062456

Identifier Type: NIH

Identifier Source: secondary_id

View Link

ELASTIC-CF

Identifier Type: -

Identifier Source: org_study_id

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