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Completed

NCT Number: NCT03001388

Longitudinal Assessment of Transient Elastography in Cystic Fibrosis

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

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Key information

Conditions

Cystic Fibrosis Liver Disease Congenital, Hereditary, and Neonatal Diseases and Abnormalities Cystic Fibrosis Digestive System Diseases Exocrine Pancreatic Insufficiency Genetic Diseases, Inborn Infant, Newborn, Diseases Lung Diseases Pancreatic Diseases Respiratory Tract Diseases

Age range

6 year–26 year

Sex eligibility

All sexes

Study type

Observational

Primary location

The Hospital for Sick Children, Toronto, Ontario, Canada

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About this study

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.

Who can participate

Healthy volunteers accepted: Yes

Only the study team can determine whether someone qualifies for participation.

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

Treatment and study plan

Transient Elastography (TE)

Procedure

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)

Primary outcomes

  1. Liver Stiffness Measurement (LSM) obtained via transient elastography using FibroScanTM

    Time frame: Baseline

    Liver stiffness" quantifies liver fibrosis and is measured in kPa (median of 10 subsequent valid measurements and are deemed acceptable if the ratio of interquartile range and median is <30% and success rate is >60%, meaning 10 valid measurements are obtained within 16 attempts).

  2. Liver Stiffness Measurement (LSM) obtained via transient elastography using FibroScanTM

    Time frame: Year 1

    Liver stiffness" quantifies liver fibrosis and is measured in kPa (median of 10 subsequent valid measurements and are deemed acceptable if the ratio of interquartile range and median is <30% and success rate is >60%, meaning 10 valid measurements are obtained within 16 attempts).

  3. Liver Stiffness Measurement (LSM) obtained via transient elastography using FibroScanTM

    Time frame: Year 2

    Liver stiffness" quantifies liver fibrosis and is measured in kPa (median of 10 subsequent

Secondary outcomes

  1. Liver steatosis obtained via transient elastography

    Time frame: Baseline, Year 1, Year 2

    Controlled Attenuation Parameter (CAP)" quantifies liver steatosis and is measured in dB

  2. Comparison of liver stiffness and liver steatosis measurements at each time point to grayscale ultrasound grades

    Time frame: Baseline, Year 1 and Year 2

    Grayscale ultrasound grades will be obtained from the parent study (PUSH: Clinical Trials: NCT01144507)

  3. Comparison of liver stiffness and liver steatosis measurements at each time point to clinical findings of portal hypertension

    Time frame: Baseline, Year 1 and Year 2

    Grayscale ultrasound grades will be obtained from the parent study (PUSH: Clinical Trials: NCT01144507)

Other outcomes

  1. Invalid measurements and success rate on liver stiffness and liver steatosis measurements

    Time frame: Baseline, Year 1 and Year 2

  2. Inter quartile range (IQR) (kPa or dB/m) of all valid measurements within the examination

    Time frame: Baseline, Year 1 and Year 2

    To reflect the dispersion of stiffness and steatosis

  3. IQR/median ratio of all valid measurements within the examination

    Time frame: Baseline, Year 1 and Year 2

    This quantity should remain as low as possible to ensure reliable results (goal < 30%)

Sponsors and collaborators

Lead sponsor

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

Nih

Collaborators

  • Cystic Fibrosis Foundation

Registry information

Important dates

Study start
2017
Primary completion
2023
Study completion
2023
First posted
Dec 23, 2016
Registry last updated
Apr 11, 2025

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

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This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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